University of Iowa · Research Skills (60:204), University of Iowa (2 lectures on grant writing), 2006-2008. Graduate Research Anatomy and Cell Biology (060:206), University of Iowa,

University of Iowa College of Medicine

Curriculum Vitae

John F. Engelhardt July 22, 2014

I. Personal Data

U.S. Citizen Name: John F. Engelhardt Marital Status: Married Spouse's Name: Susie Engelhardt Children: Three Home Address: 16 Burr Oak Court Iowa City, Iowa 52246

II. Education

1985 B.S. Iowa State University Biochemistry 1990 Ph.D. Johns Hopkins University Human Genetics

III. Post-Graduate Education

1991-92 Res. Assoc. University of Michigan, Ann Arbor HHMI 1992-93 Res. Assoc. University of Michigan, Ann Arbor Internal Medicine

IV. Academic Appointments 2006-present Department Chair, Department of Anatomy and Cell Biology, University of

Iowa, Iowa City, IA 2004-2006 Interim Department Chair, Department of Anatomy and Cell Biology, University of

Iowa, Iowa City, IA 2001-present Professor, Department of Anatomy and Cell Biology, University of Iowa, Iowa City, IA 2001-present Professor, Department of Internal Medicine, University of Iowa, Iowa City, IA 1998-present Director, Gene Therapy Center for Cystic Fibrosis and Other Genetic Diseases,

University of Iowa, Iowa City, IA 1998-present Director, Animal Models Core, Gene Therapy Center for Cystic Fibrosis and

Other Genetic Diseases, University of Iowa, Iowa City, IA 1998-2005 Director, Graduate Program in Anatomy and Cell Biology, University of Iowa,

Iowa City, IA 1997-2001 Associate Professor, Department of Anatomy and Cell Biology, University of

Iowa, Iowa City, IA

1997-2001 Associate Professor, Department of Internal Medicine, University of Iowa, Iowa City, IA

1994-1997 Assistant Professor (Joint Appt), Wistar Institute, Philadelphia, PA

John F. Engelhardt – July 22, 2014 Page 2 of 46

1996-1997 Associate Director, Graduate Gene Therapy Program, University of

Pennsylvania, Philadelphia, PA 1995-1996 Director, Cystic Fibrosis Programs at the Institute for Human Gene Therapy,

University of Pennsylvania, Philadelphia, PA 1993-1996 Director, Cellular Morphology Core, Institute for Human Gene Therapy,

University of Pennsylvania, Philadelphia, PA

1993-1997 Assistant Professor, Department of Molecular and Cellular Engineering, University of Pennsylvania, Philadelphia, PA

1992-1993 Assistant Research Scientist, Department of Internal Medicine, University of

Michigan, Ann Arbor, MI

1991-1993 Director, Cellular Morphology Core, Department of Internal Medicine, University of Michigan, Ann Arbor, MI

Other Employment Pertaining to Current Professional Appointments

None

Certification and Licensure

None Professional Affiliations

American Society for the Advancement of Science American Thoracic Society American Society for Cell Biology American Society of Gene and Cell Therapy American Society for Microbiology American Society for Biochemistry and Molecular Biology Society for the Study of Reproduction Association of Anatomy Cell Biology Neurobiology Chairpersons

V. Areas of Research Interest Research in the Engelhardt laboratory focuses on the molecular basis of inherited and environmentally

induced diseases, and on the development of gene therapies for these disorders. Included are two major

research areas: 1) the study of lung molecular and cellular biology as it relates to the pathogenesis and

treatment of cystic fibrosis (CF) lung disease, and 2) the molecular mechanism underlying redox-

mediated injury and the development of molecular therapies for ischemia/reperfusion injury, sepsis, and

the neurodegenerative disease amyotrophic lateral sclerosis (ALS). Research on lung biology includes

the study of adult epithelial stem cells and their niches in the airway, and dissecting the pathogenesis of

lung disease in animal models of cystic fibrosis. The laboratory is currently elucidating transcriptional

signals important for early establishment of the airway glandular stem cell niche, and for mobilization of

stem cells from this niche following airway injury. The newly generated ferret and pig models of cystic

fibrosis are being used to understand how stem cells in the adult airway respond to injury resulting from

dysfunction of CFTR (the chloride channel that is defective in cystic fibrosis). These new genetic models

of CF are also being used to dissect pathophysiologic mechanisms of disease and to develop gene

therapies that target the lung with recombinant adeno-associated virus. A second area of interest is the

development of pharmacologic and gene-based therapies for redox-dependent disorders of the liver and

spinal cord. Such studies are aimed at the treatment of environmentally induced liver injuries—those


resulting from sepsis, ischemia/reperfusion, and (in the case of ALS) motor neuron injury. We are using

rodent transgenic and knockout models to better understand the mechanisms that are involved in

inflammation and injury and are controlled by reactive oxygen species. Of particular interest in this area

are mechanisms of redox- ral NADPH oxidase complexes (Nox1, Nox2

and Nox4), whose roles include generating intracellular superoxides in response to extracellular pro-

inflammatory cytokine signals (IL-1-beta and TNF-alpha pathways are a major focus). This redox-

signaling program heavily utilizes recombinant viral vectors, transgenic/knockout mice, and proteomic

approaches to address basic aspects of pathophysiology, and also to generate new therapies capable of

promoting organ regeneration and repair while also minimizing the deleterious inflammatory responses to

injury.

Current NIH Funded Projects Biology of Submucosal Gland Stem Cells in the Airway (MERIT AWARD R01 Funded) Pathophysiology and Gene Therapy in a new Cystic Fibrosis Ferret Model (R01 Funded) Mechanisms of Cystic Fibrosis Related Diabetes in ferrets and children (R24 Funded)

VI. Teaching Activities MB605 Univ. of Penn (Graduate student seminar course in Molecular Biology) 1994-95. Organized a graduate/post-doc seminar series at the IHGT, 1996-present.

CAMB610 Univ. of Penn (Graduate course-Molecular Basis of Gene Therapy), 1996-97 Co-Instructed a short course in "Gene Therapy of Inherited and Acquired Disorders" at the University of Nebraska Medical Center, May 13-17, 1996.

Graduate Cell Biology I (60:216), University of Iowa (10 lecture set on Nucleus), 1997-1999. Graduate Cell Biology I (60:216), University of Iowa (6 lecture set on Nucleus), 2000-2002. Graduate Student Seminar (060:224), University of Iowa, 2002-2004 Research Skills (60:204), University of Iowa (3 lectures on grant writing), 2000-2005. Medical Cell Biology (60:116), University of Iowa (1 Lecture on Signal Transduction), 1998- present. Research Skills (60:204), University of Iowa (2 lectures on grant writing), 2006-2008. Graduate Research Anatomy and Cell Biology (060:206), University of Iowa, 1997-present.

VII. Teaching Activities Other than Classroom or Clinical

Eppley Short Course in Cancer Biology, Gene Therapy Section - Eppley Institute, Nebraska 5/1996 Advances in Genetic Modifications, Short Course - Department of Human Genetics, University of Michigan, 3/2000 University of Iowa Postdoctoral Group Meeting - Guest Speaker: “Fellowship and Grant Applications” 03/2011 University of Iowa Postdoctoral Group Meeting - Guest Speaker: “Fellowship and Grant Applications” 02/2014

VIII. Other Professional Activities

Journal Ad HOC Reviewer Journal of Clinical Investigation, American Journal of Respiratory Cell and Molecular Biology,

American Journal of Physiology: Cell Physiology, American Journal of Physiology: Gastroenterology, Gastroenterology, Nature Genetics, Molecular Therapy, Virus Research, Journal of Virology, Nature Medicine, Glycosylation and Disease, Human Gene Therapy, Gene Therapy, Molecular Cell, Journal of Infectious Disease, Nature Biotechnology, Nature, Molecular and Cellular Biology, Cell, and New England Journal of Medicine

Editorial Involvement


Antioxidant and Redox Signaling, Editor of Forum issue, 2000 Reproductive Biology and Endocrinology, Editorial Board, 2002-2003 Reproductive Biology and Endocrinology, Associate Editor, 2003-2006

Journal of Biological Chemistry, Editorial Board, 2003-2007 Antioxidant and Redox Signaling, Editorial Board, 2000-present Respiratory Research, Editorial Board, 2000-present Human Gene Therapy, Editorial Board, 2001-present Reproductive Biology and Endocrinology, Advisory Board, 2002-2007 Antioxidant and Redox Signaling, Co-Editor of Forum Issue, 2008

IX. Clinical Activities

Gene Therapy of Cystic Fibrosis Lung Disease Using E1 Deleted Adenoviruses: A Phase I Trial, PI-James M. Wilson, University of Pennsylvania. 1993 Gene Therapy for Cystic Fibrosis Using E1-Deleted Adenovirus: A Phase I Trial in the Nasal Cavity, PI-Richard C. Boucher, University of North Carolina at Chapel Hill. 1993 Gene Therapy of Cystic Fibrosis Lung Disease Using the Second Generation Adenovirus H5.110CBCFTR: A Phase I Study, PI-James Wilson, University of Pennsylvania. 1993 Oral glucose tolerance testing in cystic fibrosis children 3 months to 5 year of age, evaluation of hormonal disturbances: Clinical trial, IRB PIs-Katie Larson-Ode, Andy Norris, John Engelhardt, University of Iowa. 2012-present.

X. Collegiate, University, University Hospitals, and National Committees

Collegiate Administrative Activities and Committees Transgenic and Genome Manipulation Core Facility Advisory Committee, 2013-2016 Carver College of Medicine Cardiothoracic Surgery Headship Search Committee, 2013 Myers Professorships Review Panel, 2012 Biochemistry Department Review Committee, 2012 Interdisciplinary Graduate Program in Genetics Review Committee, 2012 Molecular and Cellular Biology Interdisciplinary Graduate Degree Program Faculty, 1997-2015 Basic Science Research Mentor for General Surgery Residents, 2011-present MSTP Enrichment and Integration Committee, 2010-present Training Grant in Virology and Viral Pathogenesis Mentor, 2010-present Carver College of Medicine Microbiology Headship Search Co-Chair, 2010 Interdisciplinary Graduate Program in Informatics Mentor (Bioinformatics Subtrack), 2010-present Carver College of Medicine Pharmacology Headship Search Committee, 2009-2010 Carver College of Medicine Neurology Headship Search Committee, 2009-2010 University of Iowa Cancer Center Internal Advisory Committee, 2009-present Carver College of Medicine Metrics Task Force, 2009-present University of Iowa Physicians Board Member, 2009-2011 (2 yr term) Carver College of Medicine Myers Teaching Professorship Committee, 2009 Carver College of Medicine Internal Medicine Headship Search Committee, 2009 Carver College of Medicine Biochemistry Headship Search Committee, 2008-2009 Carver College of Medicine LCME Accreditation Site Visit Self Study Task Force, 2008 Carver College of Medicine Information Technology Mac Desktop Support Committee, 2008 Carver College of Medicine OB/GYN Headship Search Committee, 2008 Carver College of Medicine Faculty Retreat Committee, 2006-2007 Executive Committee, MSTP Program, University of Iowa, 2006-present Core Faculty Member, Nanoscience and Nanotechnology Institute, 2006-present Biological and Physical Sciences Task Force, 2005-2006 Interdisciplinary Research and Shared Credit Task Force, 2005-2006 Steering Committee; Carver College of Medicine Strategic Planning, 2004-2005 Medical Council, CCOM, 2004-present Research Training Group, CCOM, 2004-present


Transgenic Faculty Advisory Committee, University of Iowa, 2002-2006 Gene Targeting Faculty Advisory Committee, University of Iowa, 2002-2006 Transgene and Gene Targeting Facility Advisory Committee, University of Iowa, 2000-2001 Biosciences Mentor Committee, Anatomy and Cell Biology, University of Iowa, 2000-2004 Biosciences Program Executive Committee, 2000-2007 Biosciences Graduate Program Admissions Committee, University of Iowa, 1999 Biosciences Graduate Recruitment Committee, University of Iowa, 1998, 1999 Anat. & Cell Biol Graduate Program Committee, University of Iowa, 1998-2004 Anat. & Cell Biol Graduate Program Advisory Committee, University of Iowa, 1998-2004 Anat. & Cell Biol Graduate Program Admissions Advisory Committee, University of Iowa, 1998-

present Free Radical and Radiation Biology Training Grant Mentor, 1999-present Steering Committee; Free Radical and Radiation Biology Training Grant, University of Iowa,

1999-present Co-Director, Animal Models Core of Gene Therapy Center, University of Iowa, 9/98-present Director, Graduate Program in Anatomy and Cell Biology, University of Iowa, 5/98-2003 Co-Director, Graduate Program in Anatomy and Cell Biology, University of Iowa, 2003-2006 Director, Gene Therapy Center for Cystic Fibrosis and Other Genetic Diseases, University of

Iowa, 1998-present Interdisciplinary Program Committee (Provost's office), University of Iowa, 1999 Physical Plant Committee of the COM, University of Iowa, 1998 Executive Committee, Molecular Biology Program, University of Iowa, 1998-2001 Admissions Committee, MSTP Program, University of Iowa, 1998-present Executive Committee, Biosciences Program, University of Iowa, 2001-2006 Admissions Committee, Molecular Biology Program, University of Iowa, 1998 Associate Director, Graduate Gene Therapy Program, University of Pennsylvania, 1/96-8/97 Executive Committee, Iowa Gene Therapy Center, 1997-present Director of Cystic Fibrosis Programs at the IHGT, University of Pennsylvania, 9/95-9/96 Faculty Advisor, Cellular Morphology Core, University of Pennsylvania, 10/94-8/97 Wistar Society Member, Wistar Institute, Philadelphia, Pennsylvania, 4/93-present Admissions Committee Molecular and Cellular Biology Graduate Group, University of

Pennsylvania, 11/96-1997 University of Pennsylvania Facilities Committee, Univ. of Pennsylvania, 10/94-10/96 Director, IHGT Cellular Morphology Core, University of Pennsylvania, 3/93-10/94 Director, Cellular Morphology Core, University of Michigan, Ann Arbor, 11/91-3/93 Executive Committee, Institute for Human Gene Therapy, Univ. of Penn, 6/93-12/94 Admissions Committee MCB Graduate Group, Univ. of Penn 1996, 1997

National Study Sections P30 Centers Molecular Therapy of Cystic Fibrosis Review Committee, National Institute of Diabetes and Digestive and Kidney Disease (DK) December 2011 K Award Review Panel, National Heart, Lung and Blood Institute Special Emphasis (HL) May 2011 K Award Review Panel, National Heart, Lung and Blood Institute Special Emphasis (HL) , November 2010 Program Projects Review Committee, National Institute of General Medical Sciences Special Emphasis (GM), August 2010 Review Committee, NIGMS/NIH, August 2010

Reviewer, The Wellcome Trust Grant, February 2009 Recombinant DNA Advisory Committee (OD) 2007-2011

Ad Hoc Reviewer, National Heart, Lung and Blood Institute (HLBP), September 2006 NIH Study Section, Tumor Progression and Metastasis, October 2006 Review Committee, Cystic Fibrosis Foundation Annual Grant Review, December 1999 NIH Study Section, Medical Biochemistry, 1998-2001 Review Committee, Cystic Fibrosis Foundation Annual Grant Review, December 1996 NIH Study Section, Gene Therapy to Mucosal Epithelium, April 1996

SPARX Steering Committee, Canadian Cystic Fibrosis Foundation, 1995-1999 Review Committee, Cystic Fibrosis Foundation Annual Grant Review, December 1995


Special Reviewer, NIH, Study Section/Gene Therapy Related Topics, January, 1995 Ad HOC Reviewer, RFA, Cystic Fibrosis Foundation, October, 1994 Ad HOC Reviewer, RDP, Cystic Fibrosis Foundation, August, 1994

Ad HOC Reviewer for Veterans Administration Grants, Perry Point, MD; March, 1994 Review Committee, Cystic Fibrosis Foundation Annual Grant Review, February, 1994 Scientific External Advisory Committees

American Society of Gene and Cell Therapy, Advisory Council, May 2013 – May 2018 American Society of Gene and Cell Therapy, Chair, Respiratory Tract Gene & Cell Therapy

Committee, 2013-2015 American Society of Gene and Cell Therapy, Co-Chair of the Program Committee, 2010-2011 American Society of Gene and Cell Therapy Respiratory Tract Gene & Cell Therapy Committee,

2010-2013 American Society of Gene and Cell Therapy Special Plenary Abstract Session Introducer; 2008 American Society of Gene Therapy Secretary; 2007-2010 American Society of Gene Therapy AAV Vectors Biology Abstract Review Committee

(coordinating reviewer); 2001, 2003, 2006 American Society of Gene Therapy Annual Meeting Participation as Symposium Speaker; 2002,

2003, 2006. 2010, 2011 American Society of Gene Therapy Annual Meeting Participation as Oral Abstract Chair or Co-

Chair; 2003, 2006, 2011 American Society of Gene Therapy Annual Meeting Participation as Educational Session

Speaker; 2004, 2006 American Society of Gene Therapy Embryonic/Somatic Stem Cell & Tissue Engineering Program

Committee; 2007 American Society of Gene Therapy Annual Meeting Participation as Workshop Speaker; 2005 American Society of Gene Therapy AAV Vectors Oral Abstract Session Chairman; 2005 American Society of Gene Therapy Respiratory Tract Gene Therapy Committee; 2005-2006 American Society of Gene Therapy Board of Directors; 2003-2006 American Society of Gene Therapy Late Breaking Abstracts Session Chair; 2006 American Society of Gene Therapy Meet-The-Investigator Session Speaker; 2006 American Society of Gene Therapy Meeting Presidential Lecture Abstract Review Committee;

2003 American Society of Gene Therapy Meeting Presidential Symposium Abstract Introduction; 2002,

2003 American Society of Gene Therapy Meeting "Meet-the-Investigator" Lunch (coordinator: AAV

Biology); 2002 American Society of Gene Therapy AAV Vectors Abstract Review Committee; 2002, 2005

(reviewer) Hospital for Sick Children Scientific Advisory Board, NIH SCOR in CF; 2000-2002 American Society of Gene Therapy Genetic Disease Abstract Review Committee; 2000

(reviewer) American Society of Gene Therapy Annual Meeting Participation Plenary Session Talk; 1999,

2000 American Society of Gene Therapy Nominating Committee; 1999-2002 Children's Hospital Medical Center, Cincinnati, External Advisory Committee for Cystic Williamsburg CFF Conference Planning Committee; 1996-1997, 2006 Cystic Fibrosis North American Conference Planning Committee; 1994-1997, 2005 Cystic Fibrosis North American Conference Abstract Review Committee; 1993-1999 Cystic Fibrosis RDP; 1999

XI. Honors and Awards 1985 Phi Kappa Phi Honor Society, Iowa State University 1985 High Scholarship Student Award, top 2% Iowa State University 1983-1985 Goodspeed-Hayward Undergraduate Scholarship in Biochemistry 1991-1992 Cystic Fibrosis Foundation Fellowship 1999 Philip C. and Ethel F. Ashby Lecturer, University of Oklahoma 1998-2002 Medical Biochemistry Study Section Permanent Member


2003-2006 American Society of Gene Therapy, Elected to Board of Directors 2003-2008 Elected to the Journal of Biochemistry Editorial Board 2004-present Roy J. Carver Chair in Molecular Medicine 2005 NIH MERIT Award (NIDDK) 2007-2010 Elected as Secretary, American Society of Gene Therapy 2010 Permanent Distinguished Visiting Professor, Ningxia University, China 2011 Recipient, University of Iowa Graduate College Outstanding Faculty Mentor

Award: Biological and Life Sciences 2012 2012 University of Iowa Graduate College Fall Commencement Address

2012 Liupan Mountain Friendship Award, Ningxia Foreign Experts Bureau, Ningxia, P.R.C. (Commends foreign experts for their contributions to Ningxia's economic construction)

XII. Financial Resources

Past Grant Support Sponsor: Cystic Fibrosis Foundation (PI John Engelhardt) Title: Cystic Fibrosis Postdoctoral Fellowship Role: Research Associate Effort: 100% Dates: 11/1/91 - 1/31/93 Total Support: $66,000 (Direct Costs) Sponsor: Cystic Fibrosis Foundation (Co-PI James Wilson) Title: Animal Models for Gene Therapy of Cystic Fibrosis Respiratory

Submucosal Glands Role: Principal Investigator Effort: 10% Dates: 5/1/93 - 3/4/94 Total Support: $30,000 (Direct Costs) Sponsor: Cystic Fibrosis Foundation - R720 (PI James Wilson) Title: Research and Development Program: Gene Transfer Approaches to CF Role: Principal Investigator Morphology Core Effort: 5% Dates: 5/1/93 - 3/4/94 Total Support: $52,400 (Direct Costs) Morphology Core Component Only Sponsor: NIH/NIDDK - RO1 DK 47967 (PI John Engelhardt) Title: Gene Transfer to the Respiratory Epithelium Role: Principal Investigator Effort: 25% Dates: 5/1/93 - 4/30/95 Total Support: $347,255 (Direct Costs) Sponsor: CFF #Z130 (PI John Engelhardt) Title: Evaluation of Surrogate Endpoints for Gene Therapy of CF Using

Catinonic Liposomes Role: Principal Investigator Effort: 20% Dates: 2/1/94 - 1/31/96 Total Support: $150,000 (Direct Costs) Sponsor: NIH/CF Foundation #S886 (PI James Wilson) Title: Gene Therapy of Cystic Fibrosis and Genetic Disease Center Grant


Role: Principal Investigator of Pilot-Analysis of Primary Defects in CF Respiratory Mucus

Effort: 20% Dates: 9/1/94 - 8/31/96 Total Support: $78,000 (Direct Costs)

Sponsor: NIDDK/NHLBI 1RO1 DK/HL51887 (PI John Engelhardt) Title: Biology of AAV integration in airway epithelial progenitors Role: Principal Investigator Effort: 15% Dates: 9/30/96 to 8/31/99 Total Support: $776,914 (Total Direct and Indirect Costs)

Sponsor: CFF Research Grant (PI John Engelhardt)

Title: Epitope Tagged Mucin as an Alternative Marker for CF Gene Therapy Efficacy

Role: Principal Investigator Effort: <3% Dates: 5/1/97 to 4/30/99 Total Support: $64,800 (Total Direct and Indirect Costs) Sponsor: CFF RFA (PI John Engelhardt) Title: Mechanisms of salt and fluid balance in the human cystic fibrosis airway Role: Principal Investigator Effort: <3% Dates of Support: 6/1/97 to 5/31/2000 Total Support: $324,000 (Total Direct and Indirect Costs)

Sponsor: NIH/NIDDK 2RO1 DK47967 (PI John Engelhardt) Title: Models of Submucosal Gland Development and Function Role: Principal Investigator Effort: 15% Dates: 5/1/95 to 4/30/2000 Total Support: $1,476,261 (Total Direct and Indirect Costs)

Sponsor: NIDDK 1RO1 DK51315 (PI John Engelhardt)

Title: Mechanisms and Therapy of Ischemia Reperfusion Injury in the Liver Role: Principal Investigator Effort: 10% Dates: 5/1/96 to 8/31/2000 Total Support: $733,721 (Total Direct and Indirect Costs)

Sponsor NIH 2R44 DK56526 (PI Lloyd Mitchell, Intronn, LLC) Title: Spliceosome Trans-splicing to Repair Cystic Fibrosis mRNA Role: PI of Subcontract from Intronn, LLC Effort: 0% Dates: 9/1/2000 to 8/31/2002 Total Support: $607,724 ($413, 418 Total Direct Costs; $194,306 Total Indirect Costs)

Sponsor: Genzyme Corporation Title: Pharmacologic Therapy for CF Mice Role: Principal Investigator Effort: 0% Dates: 9/26/02 to 9/25/03 Total Support: $19,883 ($16,706 Total Direct Costs; $3,177 Total Indirect Costs) Sponsor: Cystic Fibrosis Foundation (ENGELH02G0) Title: Development of a Ferret Cystic Fibrosis Model by Embryo Cloning


Role: Principal Investigator Effort: 0% Dates: 4/01/02-3/31/04 Total Support: $64,800 ($60,000 Total Direct Costs; $4,800 Total Indirect Costs) Sponsor: NIH/NHGRI CHORI-237 (PI Gregory Leno) Title: Ferret Bacterial Artificial Chromosome Library Construction Role: Co-Principal Investigator Effort: 0% Dates: 09/01/04 to 05/01/05 Total Support: 0 Sponsor: NIH/NIDDK 2RO1 DK47967 (PI John Engelhardt)

Title: Biology of Submucosal Gland Stem Cells in the Airway Role: Principal Investigator Effort: 20% Dates: 5/1/93 to 4/30/2005 Total Support: $1,653,750 (Total Direct and Indirect Costs)

Sponsor: NIDDK 2RO1 DK51315 (PI John Engelhardt)

Title: Mechanisms of Ischemia Reperfusion Injury in the Liver Role: Principal Investigator Effort: 15% Dates: 5/1/96 to 8/31/05 Total Support: $992,250 (Total Direct and Indirect Costs)

Sponsor: Cystic Fibrosis Foundation (ENGELH04G0) Title: Generating Larger Animal Models of Cystic Fibrosis Role: Principal Investigator Effort: 0% Dates: 01/01/05-02/28/06 Total Support: $ 162,000 (Total Direct and Indirect Costs) Sponsor: Cystic Fibrosis Foundation (ENGELH06G0) Title: Cloning a CFTR Knock-Out Ferret Model Role: Principal Investigator Effort: 0% Dates: 06/01/06-05/31/08 Total Support: $ 635,304 (Total Direct and Indirect Costs)

Sponsor: NIH / NHLBI 2 P50 HL061234 (PI Michael Welsh) Title: SCOR in Airway Biology and Pathogenesis of Cystic Fibrosis Role: Principal Investigator of P01 (“Generating a Ferret Model of Cystic

Fibrosis”) Effort: 20% Dates: 09/01/03-08/31/08 Total Support: $ 2,017,826 (Total Direct and Indirect Costs of Project Only)

Sponsor: NIH/HBPP 1RO1 DK067928-04 (PI John Engelhardt) Title: Redox-Mediated Mechanisms of NF-kappaB activation by LPS and IL-1 Role: Principal Investigator Effort: 20% Dates: 07/01/04-06/30/09

Total Support: $1,475,000 (Total Direct and Indirect Costs) Sponsor: NIAID contract HHSN272200900006C (PI Kerstin Lindblad-Toh) Title: Ferret Genome Sequencing Project

Role: Subcontract Principal Investigator


Effort: 0% Dates: 2/1/10 - 1/31/11 Total Support: $53,560 (Subcontract total)

Sponsor: NIDDK/NHLBI 2RO1 DK/HL58340-10 (PI John Engelhardt)

Title: Mechanisms of rAAV Transduction in the Airway Role: Principal Investigator Effort: 10% Dates: 9/30/96 to 1/31/11 Total Support: $1,843,750 (Total Direct and Indirect Costs)

Sponsor: 2 P30 DK054759-11S2 Title: Center for Gene Therapy of Cystic Fibrosis and Other Genetic Diseases

(ARRA Supplement: Pilot and Feasibility Grants) Role: Director of Center Effort: 0% Dates: 05/08/09 to 3/31/2014 Total Support: $200,000 (Total Direct and Indirect Costs)

Sponsor: NHLBI 5RC1HL099516 Title: Pathological Evaluation of a Cystic Fibrosis Ferret Model

Role: Principal Investigator Effort: 5% Dates: 9/30/09 - 8/31/11 Total Support: $998,944 (Total Direct and Indirect Costs)

Sponsor: Department of Defense, Army W81XWH-08-1-0645 Amend P2 (PI Barrie Carter

Title: Pre-Clinical Development of Therapeutics for Amyotrophic Lateral

Sclerosis Role: Co-PI Effort: 3% Dates: 05/17/2010-11/14/2011 Total Support: $2,156,733 (Total Direct and Indirect Costs)

Sponsor: 2 P30 DK054759-11S1 Title: Center for Gene Therapy of Cystic Fibrosis and Other Genetic Diseases

(Equipment Supplement) (PI John Engelhardt) Role: Director of Center Effort: 0% Dates: 05/08/09 to 3/31/2014 Total Support: $53,604 (Total Direct and Indirect Costs)

Sponsor: NIDDK R24 DK91221-01 (seeding grant) (PI John Engelhardt) Title: Etiology of Cystic Fibrosis-Related Diabetes in a CFTR-knockout Ferret Role: Principal Investigator Effort: 3.75% Dates: 07/20/2011-06/30/2012 Total Support: $ 485,396 (Total Direct and Indirect Costs)

Sponsor: NIDDK R24 DK96518 (PI John Engelhardt) Title: Early Pathogenesis of Cystic Fibrosis Related Diabetes

(Team science grant involving three institutions and a clinical trial) Role: Principal Investigator Effort: 20% Dates: 07/01/2012-06/30/2014 Total Support: $2,631,533 (Total Direct and Indirect Costs)


Sponsor: NIDDK 2RO1 DK51315-15 (PI John Engelhardt) Title: Redox Mechanisms of Ischemia Reperfusion Injury in the Liver Role: Principal Investigator Effort: 11.25% Dates: 4/1/09 to 3/31/14 Total Support: $1,875,000 (Total Direct and Indirect Costs)

Pending Grant Support Sponsor: NHLBI 1R24HL123482-01 Title: National Ferret Resource and Research Center on Lung Disease Role: Principal Investigator

Effort: 15% Proposed Dates: 6/1/14 to 5/31/2019 Total Proposed Support: $3,872,386 (Total Direct and Indirect Costs)

Sponsor: NIH-PPG HL051670 Title: Gene Therapy for Cystic Fibrosis Lung Disease Role: Principal Investigator of Animal Core

Effort: 3% Proposed Dates: 8/1/14 to 7/31/2019 Total Proposed Support: $2,416,000 (Total Direct and Indirect Costs for Core only)

Sponsor: NIH-PPG HL051670 Title: Gene Therapy for Cystic Fibrosis Lung Disease Role: Principal Investigator of P01

Effort: 20% Proposed Dates: 8/1/14 to 7/31/2019 Total Proposed Support: $1,523,000 (Total Direct and Indirect Costs for PO1 only)

Present Grant Support

Sponsor: NIH/NIDDK 4R37 DK47967-24 (PI John Engelhardt) Title: Biology of Submucosal Gland Stem Cells in the Airway–MERIT AWARD Role: Principal Investigator Effort: 15% Dates: 5/1/93 to 6/30/2015 (Second cycle of MERIT) Total Support: $2,127,418 (Total Direct and Indirect Costs) Sponsor: NIH/NIDDK P30 DK54759-15 (PI John Engelhardt) Title: Core Center for Gene Therapy of Cystic Fibrosis and Other Genetic Diseases Role: Director of Center (PI Administrative Core and Animal Models Core) Effort: 20% Dates: 05/08/09 to 3/31/2014 Total Support: $5,625,000 (Total Direct and Indirect Costs)

Sponsor: NHLBI 1R01HL108902-03 (PI John Engelhardt) Title: rAAV-directed gene therapy to the lung in cystic fibrosis ferret

models Role: Principal Investigator Effort: 7.5% Dates: 07/01/2011-04/30/2015 Total Support: $ 1,844,125 (Total Direct and Indirect Costs)


Sponsor: NIDDK R24 DK96518 Title: Early Pathogenesis of Cystic Fibrosis Related Diabetes Role: Principal Investigator

Effort: 20% Proposed Dates: 7/1/14 to 6/30/2019 Total Support: $7,515,080 (Total Direct and Indirect Costs)

Present/Past Fellowships/Mentored Grants Sponsor: CFF Post-Doctoral Research Fellowship Grant (PI Qinshi Jiang) Title: Mechanisms of ATP Transport Facilitated by CFTR Role: Sponsor Dates: 2/1/97 to 1/31/99 Total Support: $68,000 (Total Direct and Indirect Costs)

Sponsor: CFF RDP Fellowship (PI-Prerna Sharma) Title: Mechanism of AAV Transduction Role: Sponsor Dates: 12/1/97 to 11/31/99 Total Support: $56,000 (Total Costs) Sponsor: Bioscience Initiative Fellowship (PI-Ziying Yan) Title: Novel Viral Intermediates as Vehicles for Drug Delivery Role: Sponsor Dates: 5/98-4/99 Total Support: $30,000 (Total Costs) Sponsor: NIH (Institutional NRSA) (PI-Salih Sanlioglu) Title: Molecular and Cellular Biology of the Lung Role: Sponsor Dates: 7/1/96-6/30/01

Sponsor: AHA Research Heartland Affiliate Postdoctoral Fellowship (PI-Jusan Yang)

Title: Gene Therapy for Redox Mediated Ischemia/Reperfusion Injury in the Heart

Role: Sponsor Dates: 7/1/00-6/30/02 Sponsor: NIH (Institutional NRSA) (PI-Fredrick Oakley) Title: Redox Mediated Signaling in the Toll-like Receptor Family Role: Sponsor Dates: 08/01/07-07/31/11 Total Support: $120,966 (Total Costs) Sponsor: NIH (Institutional Research Fellowship Kidney Disease, Hypertension and Cell Biology) (PI-Nicholas Keiser) Title: Institutional Research Fellowship Kidney Disease, Hypertension and Cell Biology Role: Sponsor Dates: 07/01/07-06/30/08 Sponsor: NHLBI USPHS Pulmonary Training Program (PI-Ryan Driskell) Title: USPHS Pulmonary Training Program Role: Sponsor Dates: 06/01/02-05/31/05


Sponsor: NHLBI USPHS Pulmonary Training Program (PI-Gary Hunninghake) Title: USPHS Pulmonary Training Program (Trainee: Merri Huggins) Role: Sponsor Dates: 07/01/07-06/30/08 Sponsor: NHLBI USPHS Pulmonary Training Program (PI-Gary Hunninghake) Title: USPHS Pulmonary Training Program (Trainee: John Fisher) Role: Sponsor Dates: 05/01/08-04/30/09 Sponsor: NHLBI USPHS Pulmonary Training Program (PI-Gary Hunninghake) Title: USPHS Pulmonary Training Program (Trainee: Paul Kaminsky) Role: Sponsor Dates: 05/01/08-04/30/09 Sponsor: NIH T32 HL07734 Title: Program in Hematology: Molecular and Cell Biology of Blood Cells (PI Steven Lentz) Role: Mentor Dates: 07/01/06-06/30/09 Sponsor: NIH 3 UL1 RR024979-04S1

Title: Epithelial Stem Cells in a Lung Transplant Model (PI Kalpaj Parekh) Role: Mentor Dates: 12/01/2009-05/31/2010 Sponsor: NICHD-NCI Title: Reproductive Scientist Development Program (PI Michael Goodheart) Role: Mentor Dates: 07/01/07-06/30/11 Sponsor: CFF KEISER08F0 Title: Adeno-Associated Virus Trafficking in the Airway Epithelium (PI Nicholas Keiser) Role: Mentor Dates: 07/01/08-06/30/11 Sponsor: NIH 1 K01 DK083429 Title: Spatial Control and Function of Nuclear Superoxide in LPS-mediated Cell Signaling Adeno-Associated Virus Trafficking in the Airway Epithelium (PI Netanya Spencer) Role: Mentor Dates: 03/01/2010-02/28/2014 Sponsor: NIH 1 F30 HL103147 Title: Mechanisms of Integrin-Linked Transduction by Recombinant Adeno- Associated Virus (PI Paul Kaminsky) Role: Mentor Dates: 07/02/2010-07/01/2013

Sponsor: NIH 1 K08 DK092284 Title: Pathobiology of Cystic Fibrosis-Related Diabetes in a Ferret Model

(PI Zoe Stewart) Role: Mentor Dates: 07/01/2011-06/30/2015

Sponsor: NIH 1 K08 HL114725 Title: Epithelial Stem Cells in Lung Transplantation


(PI Kalpaj Parekh) Role: Mentor Dates: 08/01/2012-04/30/2016

Sponsor: NIH T32GM082729 Title: Bioinformatics Training in the Life Sciences and Biomedicine

(Trainee: Scott Tyler) Role: Mentor Dates: 07/01/2012-06/30/2013

XIII. Physical Facilities

1-111 BSB – 2,500 sq.ft. 1-100 BSB – 2,000 sq. ft. 1-366 BSB – 500 sq. ft.

XIV. Staff Personnel Currently Supervised

Yulong Zhang (Research Specialist III) Weihong Zhou (Senior Research Assistant) Meihui Luo (Research Assistant) Yaling Yi (Research Assistant) Julie Stark (Administrative Services Coordinator) Bo Liang (Research Assistant) Ziying Yan (Associate Research Scientist) Xingsheng Sun (Associate Research Scientist) Idil Apak (Assistant Research Scientist) Xiaoyan Wang (Research Assistant) Michael Winter (Assistant Research Scientist)

XV. Masters’ and Ph.D. Theses Directed and Postdoctoral Fellows Supervised

Graduate Group Involvement: Graduate Program in Cell and Molecular Biology, University of Pennsylvania, 1994-1997 Graduate Program in Pathology, University of Pennsylvania, 1994-1997 Graduate Program in Molecular Biology, University of Iowa, 1997-present Graduate Program in Anatomy and Cell Biology, University of Iowa, 1997-present Graduate Program in Translational Medicine, University of Iowa, 1997-present MSTP Program, University of Iowa, 1999-present Comprehensive Exam Committees: Xinping Yue, Anatomy and Cell Biology Graduate Program, University of Iowa, 1997

Hong Wang, Radiation Biology Graduate Program, University of Iowa, 1999 Weiling Zhao, Radiation Biology Graduate Program, University of Iowa, 1999-2000 Terri K. Kenyon, Molecular Biology Graduate Program, University of Iowa, 1999 Jason Duex, Molecular Biology Graduate Program, University of Iowa, 2000 Rebecca Oberley, Anatomy and Cell Biology Graduate Program, University of Iowa, 2001 Matthew Zimmerman, Anatomy and Cell Biology Graduate Program, University of Iowa, 2002 Shawn Hingtgen, Anatomy and Cell Biology Graduate Program, University of Iowa, 2002 Timothy Lindley, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2002 Christine Weydert, Radiation Research, University of Iowa, 2002 Rana Dajani, Molecular Biology Graduate Program, University of Iowa, 2002 Matthew Stern, Anatomy and Cell Biology Graduate Program, University of Iowa, 2003 Wei Li, Genetics Graduate Program, University of Iowa, 2004 Alok Shah, Molecular Biology Graduate Program, University of Iowa, 2006 Alix Ashare, Department of Internal Medicine, University of Iowa, 2006


David Infanger, Anatomy and Cell Biology Graduate Program, University of Iowa, 2006 Jinsil Kim, Anatomy and Cell Biology Graduate Program, University of Iowa, 2008 Jesse Riordan, Anatomy and Cell Biology Graduate Program, University of Iowa, 2009 Jennifer Delfs, Anatomy and Cell Biology Graduate Program, University of Iowa, 2009 Amy Yung Guimaraes, Anatomy and Cell Biology Graduate Program, University of Iowa, 2012 Shivangi Inamdar, Molecular and Cellular Biology, University of Iowa, 2012 Wenjie Yu, Anatomy and Cell Biology Graduate Program, University of Iowa, 2014 Thesis Committees of Students From Other Laboratories Jun Luo, Anatomy & Cell Biology Graduate Program, Univ. of Iowa, 1998-1999 Kavita Khubchandani, Anatomy & Cell Biology Graduate Program, Univ. of Iowa, 1999-2000 Weiling Zhao, Radiation Biology Graduate Program, Univ. of Iowa, 1999-2000 Branimir Cvetkovic, Molecular Biology Graduate Program, University of Iowa, 1999-2001

Jason Duex, Molecular Biology Department Graduate Program, Univ. of Iowa, 2001-2004 Shawn Hingtgen, Anatomy & Cell Biology Dept. Graduate Program, Univ. of Iowa, 2002-2004 Suneer Jain, Anatomy & Cell Biology Dept. Graduate Program, Univ. of Iowa (M.S.) 2001-2004

Rebecca Oberley, Anatomy & Cell Biology Dept. Graduate Program, Univ. of Iowa, 2002-2004 Matthew Zimmerman, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2001-2004 Timothy Lindley, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2001-2004

Christine Darbey, Radiation Biology Graduate Program, Univ. of Iowa, 2002-2004 Matthew Stern, Anatomy and Cell Biology Graduate Program, University of Iowa, 2004-2007 Stacey Payne, Anatomy and Cell Biology Graduate Program, University of Iowa, 2004-2006

Wei Li, Genetics Graduate Program, University of Iowa, 2004-2008 Alok Shah, Molecular Biology Graduate Program, University of Iowa, 2006-2009 Andrea Flynn, Anatomy and Cell Biology Graduate Program, University of Iowa, 2005-2008 Joshua Eastvold, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2005-2005 David Infanger, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2006-2009 Christian Overgaard, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2007-2009

Alix Ashare, Department of Internal Medicine, University of Iowa, April, 2008 Jinsil Kim, Anatomy and Cell Biology Graduate Program, University of Iowa, 2008-2012 Xiao Li, Anatomy and Cell Biology Graduate Program, University of Iowa, 2012-2013 Jesse Riordan, Anatomy and Cell Biology Graduate Program, University of Iowa, 2009-2013 Jennifer Delfs, Anatomy and Cell Biology Graduate Program, University of Iowa, 2009-2014 Shivangi Inamdar, Molecular and Cellular Biology Graduate Program, University of Iowa, 2012-

Thesis Level Predoctoral Graduate Students in the Engelhardt laboratory: Scott Tyler, Molecular and Cellular Biology Graduate Program, University of Iowa, 2012- Thomas Lynch, Anatomy and Cell Biology Graduate Program, University of Iowa, 2010- Paul Kaminsky (MSTP), Anatomy & Cell Biology Graduate Program,University of Iowa,2007-2012 Weam Shahin, Anatomy and Cell Biology Graduate Program, University of Iowa, 2008- John Fisher, Anatomy and Cell Biology Graduate Program, University of Iowa, 2007-2012 Weiliang Xie, Molecular Biology Graduate Program, University of Iowa, 2006-2012 Fredrick Oakley (MSTP), Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2005-2010 Merri (Ashleigh) Huggins, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2007-

2008 (Dropped out of Program) Maged Harraz, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2001-2006 Jennifer Marden, Molecular and Cellular Biology Graduate Program, Univ. of Iowa, 2001-2007 Liang (Neal) Zhang, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2002-2006 Ryan Driskell, Molecular Biology Graduate Program, Univ. of Iowa, 2002-2006 Rana Dajani, Molecular Biology Graduate Program, Univ. of Iowa, 2003-2005 Wei Ding, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2000-2005 Qiang Li, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 1999-2003 Chongxia Zhong, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 2000-2002

(Graduated with Masters) Chenguang Fan, Anatomy and Cell Biology Graduate Program, Univ. of Iowa, 1998-2002 Jeff Halldorson, Cell Biology Graduate Program, University of Pennsylvania, 1996-1997,

(transferred to different laboratory when Engelhardt moved to Univ. of Iowa)


Xiarong Wang, Cell Biology Graduate Program, University of Pennsylvania, 1996-1997 (transferred to different laboratory when Engelhardt moved to Univ. of Iowa)

Dongsheng Duan, Cell Biology Graduate Program, University of Pennsylvania, 1994-1997 Masters Students in the Engelhardt laboratory:

Duane Abbott, 2008-2009 (Master’s Thesis level) Chongxia Zhong, 2000-2002 (Master’s Thesis Level)

Rotation Predoctoral Graduate Students in the Engelhardt laboratory: Rebecca Autenried (MSTP), University of Iowa, 2014 Pavana Rotti, UI Biomedical Engineering, 2014 Shehab Ahmed, University of Iowa, 2012 Adrianne Crooke, University of Iowa, 2012 Scott Tyler, University of Iowa, 2011 Gilbert (Van) Schaeffer, II, University of Iowa, 2011 Gohar Manzar, University of Iowa, 2010 Thomas Lynch, University of Iowa, 2010 Pavla Brachova, University of Iowa, 2009

Weam Shahin, University of Iowa, 2008 Madeleine Stump, University of Iowa, 2008 Paul Kaminsky, (MSTP) University of Iowa, 2007 Theodore Wilson, University of Iowa, 2007 Eric Kaiser, (MSTP) University of Iowa, 2007 Merri (Ashliegh) Huggins, University of Iowa, 2007 John Fisher, University of Iowa, 2006 Justin Schwartz, University of Iowa, 2006 Weiliang Xie, University of Iowa, 2006 Jaime Sabel, University of Iowa, 2005 Jennifer Delfs, (MSTP) University of Iowa, 2005

Fredrick Oakley, (MSTP) University of Iowa, 2004 Michael Hitchler, University of Iowa, 2004 Ryan Boundreau, University of Iowa, 2002 Liang Zhang, University of Iowa, 2002 Jennifer Brown, University of Iowa, 2000 Yijun Chen, University of Iowa, 2000

Maged Harraz, University of Iowa, 2000 Xiaoxia Xio, University of Iowa, 2000 Rebecca Oberley, University of Iowa, 2000 Shawn Hingshen, University of Iowa, 2000 Wei Ding, University of Iowa, 1999

Jason Deux, University of Iowa, 1999 Dong Song, University of Iowa, 1999

Tim Barrett, University of Iowa, 1998 Yehong Ye, University of Iowa, 1996 Darcie Sharp, University of Iowa, 1995 Qiang Chang, University of Iowa, 1995 Jie Yao, University of Iowa, 1994 Terry Gouthro, University of Iowa, 1994

Medical Student in the Research Distinction Track in the Engelhardt laboratory: Jamie Tjaden, Summer 2010

Nam Tran, 2000-2004

Postdoctoral Students in the Engelhardt laboratory: Weiliang Xie, Ph.D., 08/12-present Hongshu Sui, Ph.D., 02/09-02/14 Nicholas Keiser, Ph.D., 12/06-07/14 Aritra Pal, Ph.D., 2009-2011


Idil Apak-Evans, 11/08-08/09 Davis Mumbengegwi, 07/04-12/08 Maged Harraz, 08/06-08/07 Xingshen Sun, 11/03-2006 Yumao Huang, 09/03-10/04 Chenguang Fan, 6/03-12/03

Xiaoming Liu, D.V.M., Ph.D., M.S., 5/00-2003 Mohammed Filali, Ph.D., 6/99-2004 Ziyi Li, Ph.D., 03/99-2001 Ningli Cheng, Ph.D., 1/99-2001 Weihong Zhou, M.D., 7/97-2001 Jusan Yang, M.D., 8/97-01/03 Ziying Yan, M.D., Ph.D., 6/97-2001 Salih Sanlioglu, D.V.M., Ph.D., 5/97-2001 Prerna Sharma, Ph.D., 9/96-1/99 Qinshi Jiang Ph.D., 1/96-7/97 Ralf Zwacka Ph.D., 4/95-7/97 Anil Sehgal Ph.D., 5/94-1/96

Undergraduates (current only): Daniel Burnette, Keegan Walters, Kael Hoflen, Attilina Scioneaux, Alec Bunting Undergraduates (past – University of Pennsylvania) Michael Hjelmstad, Micaela Hosch, Terese Zidon Undergraduates (past – University of Iowa) Duane Abbott, Tim Eggleston, Suneer Jain, Kelly Goodwin, Nick Heiniger, Amie Lang, Kyle O'Johnson, Amanda Overstreet, Ansgar S. Olsen, Tammy Reavis, Dan Ross, Li Tang, Josh TeSlaa, Andrea Park, Christopher Moothart, Benjamin Cates, Drew Arnold, Lindsey Arnold, Matthew Bartucci, Ashley Brown, Lindsey Frost, Kathryn Nelson, Rachel Smith, Brad Smyser, Kelly Deatsch, Chris Olesen, Jonathan Hyland, Barbara Hall, Yi Ou, Elizabeth Stroebele, Scott Shields, Branko Miljkovic, David Kusner, Evan Schwarz, Scott Shields, Kelly Deatsch, Kyleigh Kriener, Ben Lee, Chao Wang, Deeksha Chada Summer High School Student:

-Stephanie Choi, 2008 (Semifinalist - Intel Science Talent Search 2009; 2

nd Place - Connecticut Science Challenge 2009)

Interdisciplinary Summer Undergraduate Research Program David Kusner, Drake University, Ames Iowa Summer Research Fellowship Joseph Nellis, pre M-1 University of Iowa Benjamin Mathews, M1, University of Iowa Research Scientists and Research Investigators:

-Dongsheng Duan, Ph.D., 7/97-2002 -Terry Ritchie, Ph.D., 7/97-2001 -Qinshi Jiang, Ph.D., 6/98-2001

-Chen-hong Guo (Visiting Scientist), 2006-2007 -Ziyi Li, Ph.D. 2002-2006 -Qiang Li, Ph.D., 2004-2011 -Xiaoming Liu, Ph.D., 2003-2014 -Xingshen Sun, Ph.D., 2006-present -Ziying Yan, Ph.D., 3/00-present -Netanya Spencer, M.D., Ph.D., 2009-2014 -Idil Apak-Evans, Ph.D., 08/09-present


-Michael Winter, Ph.D. 01/12-present

Medical Fellows and Clinical Assistant Professors: -Carl Williams, M.D., 7/99-2002

-Peter Benson, M.D., 9/99-2001 -Netanya Spencer, M.D., Ph.D., 2006-2009 -Michael Goodheart, M.D., 2005-2010 -Kalpaj Parekh, M.D., 2008-present -Zoe Stewart, M.D., Ph.D., 2010-present

Summary of trainees: In my 17 years as a faculty member, I have hosted/mentored 24 postdoctoral scholars and M.D. fellows, 31 rotating predoctoral graduate student, 19 thesis level predoctoral graduate students, and three clinical assistant professors in my laboratory. Of the 19 predoctoral students, 5 are still completing their thesis in my laboratory, 2 did not graduate from the Ph.D. program and are technicians, 1 has unknown whereabouts, 1 is completing medical residency training and was in the MSTP, 4 are currently in postdoctoral positions, 4 are in tenure track faculty positions, and 2 are in tenured faculty positions. Of the 24 postdoctoral students who have trained in my laboratory, 3 are still in training in my laboratory, 3 are currently in second post-doc positions, 4 are working in the biotech sector, 1 has left science, 1 is a lab manager in my laboratory, 2 have gone onto complete their medical residencies, and 10 are in faculty positions (3 are in non-tenure track faculty positions, 3 are in tenure track faculty positions, and 4 are in tenured faculty positions). Of the 3 clinician scientists I have mentored, 1 has his own funded laboratory at University of Iowa and is funded, and 2 are still being mentored in my laboratory.

XVI. Bibliography Peer Reviewed Journals

1. Mitchell GA, Looney JE, Brody LC, Steel G, Suchanek M, Engelhardt JF, Willard HF and

Valle D: Human ornithine--aminotransferase: cDNA cloning and analysis of the structural gene. J Biol Chem 263:14288-14295, (1988).

2. Mitchell GA, Brody LC, Sipila I, Looney JE, Wong C, Engelhardt JF, Patel AS, Steel G, Obie

C, Kaiser-Kupfer MI and Valle D: At least two mutant alleles of ornithine--aminotransferase cause gyrate atrophy of the choroid and retina in Finns. Proc Natl Acad Sci USA 86:197-201, (1989).

3. Raj BN, Engelhardt JF, Au W-C, Levy ED and Pitha PM: Virus infection and interferon can

activate gene expression through a single synthetic element but endogenous genes show distinct regulation. J. Biol. Chem. 264:16658-16666, (1989).

4. Mitchell GA, Brody LC, Engelhardt JF, Steel G, Obie C, Fontaine G, Saudubray J-M,

Bonnefont J-P, Labuda D and Valle D: Inactivation of ornithine--aminotransferase by splice mediated insertion of Alu sequences: A new role for Alu in humans mutations? Ped Res 24, (1990).

5. Engelhardt JF, Bisat F, Kellum MJ, and Pitha PM: Retrovirus Vector-Targeted inducible

expression of Human -Interferon Gene to B-Cells. Virology 178:419-428, (1990). 6. Engelhardt JF, Steel G and Valle D: Transcriptional analysis of ornithine aminotransferase

gene promoter. J. Biol. Chem. 266:752-758, (1991).

7. Engelhardt JF, Allen E and Wilson JM: Reconstitution of tracheal grafts with a genetically modified epithelium. PNAS 88:11192-11196, (1991).

8. Whitsett JA, Dey C, Wikenheiser KA, Clark JC, Wert ES, Gregory RJ, Smith AE, Cohn

JA,Wilson JM, and Engelhardt JF: Human cystic fibrosis transmembrane conductance


regulator directed to respiratory epithelial cells of transgenic mice. Nature Genetics 2:13-20, (1992).

9. Engelhardt JF, Yankaskas JR, Ernst SA, Yang Y, Marino CR, Boucher RC, Cohn JA and

Wilson JM: Submucosal glands are the predominant site of CFTR expression in the human bronchus. Nature Genetics 2:240-248, (1992).

10. Engelhardt JF, Yankaskas JR, and Wilson JM: In vivo retroviral gene transfer into human

bronchial epithelia of xenografts. .J Clin. Inv. 90:2598-2607, 1992. 11. Engelhardt JF, Yang Y, Stratford-Perricaudet LD, Allen ED, Kosarsky K, Perridaudet M,

Yankaskas JR and Wilson JM: Direct gene transfer of human CFTR into human bronchial epithelia of xenografts with E1 deleted adenoviruses. Nature Genetics 4:27-33, (1993).

12. Yang Y, Raper SE, Cohn JA, Engelhardt JF and Wilson JM: An approach for treating the

hepatobillary disease of cystic fibrosis by somatic gene transfer. Proc Natl Acad Sci USA 90:4601-4605, (1993).

13. Yang Y, Devor DC, Engelhardt JF, Ernst SA, Strong TV, Collins F, Cohn JA, Frizzell RA and

Wilson JM: Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR. Human Molecular Genetics 2:1253-1261, (1993)

14. Engelhardt JF, Simon RH, Yang Y, Zepeda M, Pendleton SW, Doranz B, Grossman M and

Wilson JM: Adenovirus-Mediated Transfer of the CFTR Gene to Lung of Non-human Primates: Biological Efficacy Study. Human Gene Therapy, 4:759-769, (1993).

15. Simon RH, Engelhardt JF, Yang Y, Zepeda M, Pendleton SW, Grossman M and Wilson JM:

Adenovirus-Mediated Transfer of the CFTR Gene to Lung of Non-human Primates: Toxicity Study. Human Gene Therapy, 4:771-780, (1993).

16. Engelhardt JF, Zepeda M, Cohn JA, Yankaskas JR and Wilson JM: Expression of the cystic

fibrosis gene in adult human lung. J. Clin. Inv., 93:737-749, (1994). 17. Grossman M, Raper SE, Kozarsky K, Stein EA, Engelhardt JF, Muller D, Lupien PJ, and

Wilson JM: Successful ex vivo gene therapy directed to liver in a patient with familial hypercholesterolaemia, Nature Genetics. 6:335-341, (1994).

18. Wilson JM, Engelhardt JF, Grossman M, Simon RH, and Yang Y. Gene Therapy of Cystic

Fibrosis Lung Disease Using E1 Deleted Adenoviruses: A Phase I Trial. Human Gene Therapy, 5:501-519 (1994).

19. Boucher RC, Knowles MR, Johnson LG, Olsen JC, Pickles R, Wilson JM, Engelhardt JF,

Yang Y, and Grossman M. Gene Therapy for Cystic Fibrosis Using E1-Deleted Adenovirus: A Phase I Trial in the Nasal Cavity. Human Gene Therapy, 5:615-639 (1994).

20. Engelhardt JF, Ye X, Doranz B, and Wilson JM: Ablation of E2A in recombinant

adenoviruses improves transgene persistence and decreases inflammatory response in mouse liver, PNAS, 91;6196-6200 (1994).

21. Yang Y, Engelhardt JF, and Wilson JM: Ultrastructural localization of variant forms of CFTR

in human bronchial epithelia of xenografts, Am. J. Resp. Cell and Mol. Biol., 11;7-15 (1994). 22. Yang Y, Nunes FA, Berencsi K, Gonczol E, Engelhardt JF and Wilson JM. Inactivation of

E2a in Recombinant Adenoviruses Improves the Prospect or Gene Therapy of Cystic Fibrosis. Nature Genetics, 7;362-369 (1994).


23. Engelhardt JF, Smith SS, Allen E, Yankaskas JR, Dawson DC and Wilson JM: Coupled secretion of chloride and mucus in skin of Xenopus laevis: possible role for CFTR Amer J Physiol: Cell, 267;C491-C500, (1994).

24. Engelhardt JF, Litsky L, and Wilson JM. Prolonged Transgene Expression in Cotton Rat

Lung with Recombinant Adenoviruses Defective in E2a. Human Gene Therapy, 5;1217-1229 (1994).

25. Grubb BR, Pickles RJ, Ye H, Yankaskas JR, Vick RN, Engelhardt JF, Wilson JM, Johnson,

LG,and Boucher, RC: Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans. Nature 371;802-806 (1994)

26. Root KV, Engelhardt JF, Post M, Wilson JM, and Van Dyke RW, CFTR does not alter

acidification of L cell endosomes. Biochem Biophys. Res Comm. 205;396-401, (1994). 27. Pilewski, JM., Engelhardt JF., Bavaria JE., Kaiser LR., Wilson JM., and Albelda S.M:

Adenovirus-mediated gene transfer to human bronchial submucosal glands using xenografts. Amer. J. Physiol: Lung Cell and Mol Physiol. 12:L657-L665, (1995)

28. Engelhardt JF, Schlossberg H, Yankaskas JR, and Dudus L. Progenitor cells of the adult human airway involved in submucosal gland development. Development 121:2031-2046 (1995).

29. Goldman MJ., Litzky LA., Engelhardt JF., and Wilson JM. Transfer of the CFTR Gene to the

Lung of Nonhuman Primates with E1-Deleted, E2a-Defective Recombinant Adenovirus: A Preclinical Toxicology Study. Human Gene Therapy 6:839-851 (1995).

30. Knowles MR., Hohneker KW., Zhou RN., Olsen J., Hoah TL., Hu PC., Leigh MW.,

Engelhardt JF., Edwards LJ., Jones KR., Grossman M., Wilson JM, Johnson LG., and Boucher RC. A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis. New England Journal of Medicine 333:823-31 (1995).

31. Zhang Y, Doranz B, Yankaskas JR, and Engelhardt JF: Genotypic analysis of respiratory

mucous sulfation defects in cystic fibrosis. J. Clin. Inv. 96:2997-3004 (1995). 32. Zhang Y, Yankaskas J, Wilson JM, and Engelhardt JF: In vivo Analysis of Fluid Transport in

Cystic Fibrosis Airway Epithelia of Bronchial Xenografts. Am. J. Physiology: Cell Physiology 270:C1326-C1335 (1996).

33. Schlossberg H, Zhang Y, Dudus L, and Engelhardt JF: Expression of c-fos and c-jun During

Hepatocellular Remodeling Following Ischemia Reperfusion in Mouse Liver Hepatology 23;1546-1555 (1996).

34. Sehgal A, Presente A, Dudus L, and Engelhardt JF: Isolation of differentially expressed

cDNAs during ferret tracheal development: Application of Differential Display PCR. Experimental Lung Research 22;419-434 (1996).

35. Sehgal A, Presente A, and Engelhardt JF: Developmental Expression Patterns of CFTR in

Ferret Tracheal Surface Airway and Submucosal Glands Am. J. Resp. Cell. and Mol. Biol. 15;122-131 (1996).

36. Presente A, Sehgal A, Dudus L, and Engelhardt J: Differentially Regulated Epithelial

Expression of an Eph Family Tyrosine Kinase (Fek2) During Tracheal Surface Airway and Submucosal Gland Development Am. J. Resp. Cell. and Mol. Biol. 16;53-61 (1997).

37. Duan D, Fisher K, Burda J, and Engelhardt JF: Structural and Functional Heterogeneity of

Integrated Recombinant AAV Genomes. Virus Research 48;41-56 (1997).


38. Zwacka R, Zhang Y, Halldorson JB, Schlossberg H, Dudus D, and Engelhardt JF: CD4

+ T-

Lymphocytes Mediate Ischemia/Reperfusion-Induced Inflammatory Responses in Mouse Liver. J. Clin. Inv. 100;279-289 (1997).

39. Bennett J, Duan D, Engelhardt J, and Maguire AM: Real-time Non-Invasive in vivo

Assessment of Adeno-Associated Virus Mediated Retinal Transduction. Invest. Opthom. and Visual Sci. 38:2857-2863 (1997).

40. Lam E, Zwacka R, Engelhardt JF, Davidson B, Domann F, and Oberley LW: Adenoviral-

Mediated Manganese superoxide dismutase gene transfer to hamster cheek pouch carcinoma cells. Cancer Research 57:5550-5556 (1997).

41. Sharma P, Dudus L, Nielsen PA, Clausen H, Yankaskas JR, Hollingworth MA, and

Engelhardt JF: MUC5B and MUC7 are Differentially Expressed in Mucous and Serous Cells of Submucosal Glands in Human Bronchial Airways. Am. J. Resp. Cell. Mol. Biol. 19:30-37 (1998). (Cover Image)

42. Duan D, Sehgal A, Yao J, and Engelhardt JF: The Lef1 transcription factor expression

defines airway progenitor cell targets for in utero gene therapy of submucosal gland in cystic fibrosis. Am. J. Resp. Cell. Mol. Biol. 18:750-758 (1998).

43. Zwacka RM, Dudus L, Epperly MW, Greenberger JS, and Engelhardt JF: Redox Gene

Therapy Protects Human IB-3 Lung Epithelial Cells Against Ionizing Radiation-Induced Apoptosis. Human Gene Therapy 9:1381-1386 (1998).

44. Zhang Y, Jiang Q, Dudus, L, Yankaskas JR, and Engelhardt JF: Vector Specific

Complementation Profiles of Two Independent Primary Defects in Cystic Fibrosis Airways. Human Gene Therapy 9:635-648 (1998).

45. Gonzalez-Zulueta, M, Mukhin G, Zwacka RM, Engelhardt JF, Oberley LW, Dawson VL, and

Dawson TM: Manganese Superoxide dismutase protects nNOS Neurons from NMDA and Nitric Oxide Mediated Neurotoxicity. J. Neuroscience 18:2040-2055 (1998).

46. Epperly M, Bray J, Kraeger S, Zwacka R, Engelhardt JF, Travis E, and Greenberger J:

Prevention of Late Effects of Irradiation Lung Damage by Manganese Superoxide Dismutase Gene Therapy. Gene Therapy 5:196-208(1998).

47. Fang X, Weintraub NL, Rios CD, Chappell DA, Zwacka RM, Engelhardt JF, Oberley LW,

Yan T, Heistad DD, and Spector AA: Overexpression of Human Superoxide Dismutase Inhibits Oxidation of Low-Density Lipoprotein by Endothelial Cells. Circulation Research 82:1289-1297 (1998).

48. Zwacka RM, Zhou W, Zhang Z, Darby CJ, Dudus L, Halldorson J, Oberley L, and Engelhardt

JF: Redox Gene Therapy of Liver Ischemia/Reperfusion Injury Reduces AP1 and NFkB Activation. Nature Medicine 4:698-704 (1998).

49. Duan D, Sharma P, Yang J, Y Yue, Dudus L, Zhang Y, Fisher KJ, and Engelhardt JF

Circular Intermediates of Recombinant Adeno-Associated Virus have Defined Structural Characteristics Responsible for Long Term Episomal Persistence In Muscle Tissue. J. Virology 72:8568-8577 (1998).

50. Zwacka RM, Zhang Y, Halldorson J, and Engelhardt JF: Ischemia/reperfusion injury in the

liver of BALB/c mice activates AP-1 and NF-B Independently of IB Degradation. Hepatology 28:1022-1030 (1998).

51. Jiang Q, Mak D, Devidas S, Schwiebert EM, Bragin A, Zhang Y, Skach WR, Guggino WB,

Foskett JK, and Engelhardt JF: Cystic fibrosis transmembrane conductance regulator-


associated ATP release is controlled by a chloride sensor. J.Cell Biology 143(3):645-57 (1998). PMCID: 2148142

52. Ceneviva GD, Tzeng E, Hoyt DG, Yee E, Gallagher A, Engelhardt JF, Kim YM, Billiar TR,

Watkins SA, and Pitt BR. Nitric oxide inhibits lipopolysaccharide-induced apoptosis in pulmonary artery endothelial cells. Am. J. Physiol 275:L717-28 (1998).

53. Duan D, Yue Y, McCray PB, and Engelhardt JF: Polarity Influences the efficiency of

recombinant adeno-associated virus infection in differentiated airway epithelia. Human Gene Therapy 9:2761-2776 (1998).

54. Woo YJ, Zhang JC, Vijayasarathy C, Zwacka RM, Engelhardt JF, Gardner TJ, Sweeney HL.

Recombinant adenovirus-mediated cardiac gene transfer of superoxide dismutase and catalas attenuates postischemic contractile dysfunction. Circulation 19:II255-60 (1998).

55. Epperly, MW, Bray, JA, Krager, S, Berry, LM, Gooding, W, Engelhardt, JF, Zwacka, R,

Travis,EL, and Greenberger, JS Intratracheal injection of adenovirus containing the human MnSOD transgene protects athymic nude mice from irradiation-induced organizing alveolitis. Int. J. Radiation Oncology Biol. Phys. 43:169-181 (1999).

56. Duan D, Sharma P, Dudus L, Zhang Y, Sanlioglu S, Yan Z, Yue Y, Ye Y, Lester R, Yang J,

Fisher KJ and Engelhardt JF. Formation of Adeno-Associated Virus Circular Genomes is Differentially Regulated by Adenovirus E4-ORF6 and E2a Gene Expression. J. Virology 73:161-169 (1999).

57. Zhang Y and Engelhardt JF. Airway Surface Fluid Volume and Chloride Content in Cystic Fibrosis and Normal Bronchial Xenografts. Am. J. Physiol: Cell Physiol 276:C469-C476 (1999).

58. Sanlioglu S, Duan D, and Engelhardt JF. Two independent molecular pathways for rAAV

genome conversion occur following UV-C and E4orf6 augmentation of rAAV transduction. Human Gene Therapy 10:591-602 (1999).

59. Duan D, Yue Y, and Engelhardt JF: Response to “Polarity Influences the efficiency of

recombinant adeno-associated virus infection in differentiated airway epithelia”. Human Gene Therapy 10:1553-1557 (1999).

60. Sanlioglu, S, and Engelhardt JF. Cellular Redox State Alters Recombinant Adeno-

Associated Virus Transduction Through Tyrosine Phosphatase Pathways. Gene Therapy 6:1427-1437, 1999.

61. Duan, D, Yan, Z, Yue, Y, and Engelhardt, JF. Structural analysis of AAV transduction

circular intermediates. Virology 261:8-14, 1999. 62. Lam EW, Zwacka R, Seftor EA, Nieva DRC, Davidson BL, Engelhardt JF, Hendrix MJC, and

Oberley LW. Effects of antioxidant enzyme overexpression on the invasive phenotype of hamster cheek pouch carcinoma cells. Free Radical Biol. & Med. 27:572-579, 1999.

63. Yang J, Zhou W, Zhang Y, Zidon T, Ritchie T, and Engelhardt JF: Concatamerization of

Adeno-Associated Virus Circular Genomes Occurs through Intermolecular Recombination. J Virology. 73:9468-9477, 1999. PMCID: 112981

64. Duan D, Yue Y, Zhou W, Labed B, Ritchie T, Grosschedl R, and Engelhardt JF: Submucosal

Gland Development in the Airway is Controlled by Lymphoid Enhancer Binding Factor-1 (Lef-1). Development, 126:4441-4453, 1999.

65. Duan D, Li Q, Kao

A, Yue Y, Pessin

J, and Engelhardt JF: Dynamin is Required for

Recombinant Adeno-associated Virus Type 2 Infection. J Virology, 73:10371-10376, 1999.


66. Walters RW, Duan D, Engelhardt JF, and Welsh MJ. Incorporation of Adeno-Associated

Virus in a Calcium Phosphate Coprecipitate Improves Gene Transfer to Airway Epithelia In Vitro and In Vivo. J. Virology, 74:535-540, 2000.

67. Naren AP, Anke D, Cormet-Boyaka E, Boyaka PN, McGhee JR, Zhou W, Akagawa K,

Jujiwara T, Thome U, Engelhardt JF, Nelson DJ, and Kirk KL. Syntaxin 1A is Expressed in Airway Epithelial Cells Where it Modulates CFTR Cl

- Currents. J. Clin. Inv. 105:377-386,

2000. 68. Sanlioglu S, Benson P, and Engelhardt JF. Loss of ATM Function Enhances Recombinant

Adeno-Associated Virus Transduction and Integration through Pathways Similar to UV Irradiation. Virology, 268:68-78, 2000. (Cover Image)

69. Duan D, Yue Y, Yan Z, and Engelhardt JF. A New Dual Vector Approach to Enhance

Recombinant AAV Mediated Gene Expression Through Intermolecular Cis-activation. Nature Med. 6:595-598, 2000.

70. Yan Z, Zhang Y, Duan D, and Engelhardt JF. Trans-Splicing Vectors Expand the Utility of

Adeno-Associated Virus for Gene Therapy. PNAS 97:6716-6721, 2000. 71. Jiang Q, Li J, Dubroff R, Ahn YJ, Foskett, JK, Engelhardt JF, Kleyman, TR. Epithelial

sodium channels regulate cystic fibrosis transmembrane conductance regulator chloride channels in Xenopus oocytes. J. Biol. Chem. 275:13266-13274, 2000.

72. Duan D, Yue Y, Yan Z, Yang J, and Engelhardt JF. Endosomal Processing Limits Gene

Transfer to Polarized Airway Epithelia by Adeno-Associated Virus. J. Clin. Inv. 105:1573-1587, 2000. (Cover Image).

73. Lam EWN, Hammad HM, Zwacka R, Darby CJ, Baumgardner KR, Davidson BL, Oberley TD,

Engelhardt JF, and Oberley LW. Immunolocalization and adenoviral vector-mediated manganese superoxide dismutase gene transfer to experimental oral tumors. J. Dent. Res.

79:1410-1417, 2000. 74. Sanlioglu, S., P.K. Benson, J. Yang, E.M. Atkinson, T. Reynolds, and J.F. Engelhardt.

Endocytosis and nuclear trafficking of adeno-associated virus type 2 are controlled by rac1 and phosphatidylinositol-3 kinase activation. J Virol. 74:9184-9196. 2000.

75. Ritchie T, Zhou W, McKinstry E, Hosch M, Zhang Y, Nathke I, and Engelhardt JF.

Developmental expression of catenins and associated proteins during submucosal gland morphogenesis in the airway. Experimental Lung Research 27:121-141, 2001.

76. Wang X, Zhang Y, Amberson A, and Engelhardt JF. New models of the tracheal airway

define the glandular contribution to airway surface fluid and electrolyte composition. Am. J. Resp. Cell. Mol. Biol. 24:195-202, 2001.

77. Zanetti M, Zwacka RM, Engelhardt JF, Katusic Z, and O’Brien T. Superoxide anions and

endothelial cell proliferation in normoglycemia and hyperglycemia. Anterioscler Thromb Vasc Biol 21:195-200, 2001.

78. Sanlioglu S, Monick MM, Luleci G, Hunninghake GW and Engelhardt, JF. Rate limiting steps

of AAV transduction and implications for human gene therapy. Current Gene Therapy 1:137-147, 2001.

79. Engelhardt JF. Stem cells niches in the mouse airway. Am. J. Respir. Cell. Mol. Biol 24:649-

652, 2001.


80. Duan D, Yan Z, Yue Y, Ding W, and Engelhardt JF. Enhancement of Muscle Gene Delivery with Pseudotyped Adeno-Associated Virus Type 5 Correlates with Myoblast Differentiation. Journal of Virology 75(16):7662-7671, 2001. PMCID: 115001

81. Li Q, Sanlioglu S, Li S, Ritchie T, Oberley L, and Engelhardt JF. GPx-1 gene delivery

modulates NFkB activation following diverse environmental injuries through a specific subunit of the IKK complex. Antioxidant and Redox Signaling 3(3):415-432, 2001.

82. Engelhardt JF, Sen CK, and Oberley L. Redox-modulating gene therapies for human

diseases Antioxidant and Redox Signaling 3(3):341-346, 2001.

83. Zhou W, Zhang Y, Hosch MS, Lang A, Zwacka RM, and Engelhardt JF. Subcellular site of

superoxide dismutase expression differentially controls AP-1 activity in the liver and injury following ischemia/reperfusion. Hepatology 33(4):902-914, 2001.

84. Sanlioglu S, Williams C, Samavati L, Butler N, Wang G, McCray P Jr., Ritchie T.

Hunninghake G, Zandi E, and Engelhardt JF. 2001. Lipopolysaccharide Induces Rac1-

dependent Reactive Oxygen Species Formation and Coordinates Tumor Necrosis Factor-

Secretion through IKK Regulation of NF-B. J. Biol. Chem. 276(32): 30188-30198.

85. Duan D, Yue Y, and Engelhardt JF. 2001. Expanding AAV Packaging Capacity with Trans-

splicing or Overlapping Vectors: A Quantitative Comparison. Molecular Therapy 4(4):383-391.

86. Li ZY, Jiang QS, Zhang YL, Liu XM, and Engelhardt JF. 2001. Successful Production of Offspring Following Superovulation and in vitro Culture of Embryos from Domestic Ferrets. Reproduction 122:611-618.

87. Khubchandani, KR, Goss, KL, Engelhardt JF and Snyder, JM. 2001. In situ hybridization of SP-A mRNA in adult human conducting airways. Pediatr Pathol Mol Med 20(5): 349-66.

88. Liu X, Jiang Q, Mansfield SG, Puttaraju M, Zhang Y, Zhou W, Cohn JA, Garcia-Blanco MA,

Mitchell LG and Engelhardt JF. 2002. Partial correction of endogenous F508 CFTR in

human cystic fibrosis airway epithelial by spliceosome-mediated RNA trans-splicing. Nature Biotechnology 20, 47-52.

89. Yan Z, Zak R, Luxton GWG, Ritchie T, Bantel-Schaal U and Engelhardt JF. 2002.

Ubiquitination of Both Adeno-associated Virus Types 2 and 5 Capsid Proteins Affect the Transduction Efficiency of Recombinant Vectors. Journal of Virology 76(5), 2043-2053. PMCID: 135943

90. Harper S, Hauser MA, Dellorusso C, Duan D, Crawford RW, Phelps SF, Harper HA, Robinson AS, Engelhardt JF, Brooks SV and Chamberlain JS. 2002. Modular flexibility of dystrophin: Implications for gene therapy of Duchenne muscular dystrophy. Nature Medicine 8(3), 253-261.

91. Yang JQ, Buettner GR, Domann FE, Li Q, Engelhardt JF, Darby Weydert C and Oberley LW. 2002. v-Ha-ras Mitogenic Signaling Through Superoxide and Derived Reactive Oxygen Species. Molecular Cardinogenesis 33, 206-218,

92. Li Z, Jiang Q, Sabet M, Zhang Y, Ritchie TC and Engelhardt JF. Conditions for In Vitro Maturation and Artificial Activation of Ferret Oocytes. Biology of Reproduction, 66, 1380-1386, 2002.

93. Yang GS, Schmidt M, Yan Z, Lindbloom JD, Harding TC, Donahue BA, Engelhardt JF, Kotin R and Davidson BL. Virus-Mediated Transduction of Murine Retina with Adeno-Associated Virus: Effects of Viral Capsid and Genome Size. J. Virology 76:15, 7651-7660, 2002. PMCID: 136354


94. Filali, M., Cheng, N., Abbott, D., Leontiev, V., and Engelhardt, JF. Wnt-3A/beta-catenin

signaling induces transcription from the LEF-1 promoter. J. Biol. Chem. 277:36, 33398-33410, 2002.

95. Engelhardt JF. The lung as a metabolic factory for gene therapy. J. Clin. Inv. 110, 429-432,

2002. PMCID: 150422

96. Doerschug K, Sanlioglu S, Flaherty DM, Wilson RL, Yarovinsky T, Monick MM, Engelhardt JF, and Hunninghake GW. 2002. First-Generation Adenovirus Vectors Shorten Survival Time in a Murine Model of Sepsis. J. Immunol. 169:6539-6545.

97. Fan, C., Yang, J., and Engelhardt, J.F. 2002. Temporal Pattern of NFkappaB Activation Influences Apoptotic Cell Fate in a Stimuli Dependent Fashion. Journal of Cell Science 115, 4843-4853.

98. Fan C, Li Q, and Engelhardt J.F. 2003. Tyrosine phosphorylation of IkappaBalpha activates

NF-kappaB through a redox-regulated and c-Src-dependent. J. Biol. Chem., 278:3, 2072-2080.

99. Tran, N.D., Liu, X., Yan, Z., Abbote, D., Jiang, Q., Kmiec, E.B., Sigmund, C.D., and Engelhardt, J.F. 2003. Efficiency of chimeraplast gene targeting by direct nuclear injection using a GFP Recovery Assay. Molecular Therapy 7:2, 248-253.

100. Yang, J., Marden, J.L., Fan, C., Sanlioglu, S., Weiss, R.M., Ritchie, T.C., Davisson, R.L. and

J.F. Engelhardt. 2003. Genetic redox preconditioning differentially modulates AP-1 and NFkB Responses Following Cardiac Ischemia/Reperfusion Injury and Protects Against Necrosis and Apoptosis. Molecular Therapy 7:3, 341-353.

101. Duan, D., Yue, Y., and Engelhardt, J.F. 2003. Consequences of DNA-Dependent protein

kinase catalytic subunit deficiency on recombinant adeno-associated virus genome circularization and heterodimerization in muscle. J. Virology 77:8, 4751-4759. PMCID: 152118

102. Li Z, Sabet MR, Zhou Q, Liu X, Ding W, Zhang Y, Renard J-P, and Engelhardt JF. 2003.

Developmental capacity of ferret embryos by nuclear transfer using G0/G1-phase fetal fibroblasts. Biology of Reproduction 68, 2297-2303.

103. Qiao, L., Han, S.L., Fang, Y., Park, J.S., Gupta, S., Gilfor, D., Amorino, G., Valerie, K.,

Engelhardt, J.F., Grant, S., Hylemon, P.B. and Dent, P. 2003. Bile Acid Regulation of C/EBP-betta, CREB and c-Jun Function, via the ERK and JNK Pathways, Modulates the Apoptotic Responses of Hepatocytes. Mol Cell Biol 23:9, 3052-3066.

104. Xiong, S., She, H., Takeuchi, H., Han, B., Engelhardt, J.F., Barton, C.H., Zandi, E., Giulivi,

C., and Tsukamoto, H. 2003. Signaling role of intracellular iron in NF-kappaB activation. J. Biol. Chem. 278:17646-17654.

105. Ding, W., Yan, Z., Zak, R., Saavedra, M., Rodman, D.M. and Engelhardt, J.F. 2003.

Second-strand genome conversion of adeno-associated virus type 2 (AAV-2) and AAV-5 is not rate limiting following apical infection of polarized human airway epithelia. J Virology 77:13, 7361-7366. PMCID: 164830

106. Li L, Chu Y, Fink GD, Engelhardt JF, Heistad DD, Chen AF. Nov. 2003. Endothelin-1

Stimulates Arterial VCAM-1 Expression via NADPH Oxidase-Derived Superoxide in Mineralocorticoid Hypertension. Hypertension, 997-1003.


107. Li Z, and Engelhardt JF. Nov. 2003. Progress toward generating a ferret model of cystic fibrosis by somatic cell nuclear transfer. Reprod Biol Endo. 1:83. (url: http://www.rbej.com/content/1/1/83) PMCID: 280727

108. Li Z, and Engelhardt JF. Nov. 2003. New technologies for genetic manipulation and animal

models: Forum introduction [Review]. Reprod Biol Endo 1:78. (url: http://www.rbej.com/content/1/1/78) PMCID: 280722

109. Yan Z, Zak R, Zhang Y, Ding W, Godwin S, Munson K, Peluso R, and Engelhardt JF.

(2004). Distinct Classes of Proteasome-Modulating Agents Cooperatively Augment rAAV2 and rAAV5-Mediated Transduction from the Apical Surface of Human Airway Epithelia. J Virology, 78:6, 2863-2874. PMCID: 353734

110. Fan C, Li Q, Zhang Y, Liu X, Luo M, Abbott D, Zhou W and Engelhardt JF. (2004). I-kappa

B-alpha and I-kappa B-beta Possess Injury Context-Specific Functions that Uniquely Influence Hepatic NF-kappaB Induction and inflammation. JCI, 113:5, 746-755. PMCID: 351311

111. Liu X, Yan Z, Luo M, Zak R, Li Z, Driskell RR, Huang Y, Tran N and Engelhardt JF. (2004).

Targeted Correction of Single Base-Pair Mutations Using Adeno-Associated Virus Vectors Under Non-Selective Conditions. J Virology, 78:8, 4165-4175. PMCID: 374254

112. Sanlioglu AD, Karacay B, Benson PK, Engelhardt JF, and Sanlioglu S. (2004). Novel

approaches to augment adeno-associated virus type-2 endocytosis and transduction. Virus Research, 104, 51-59.

113. Liu X, Driskell RR, Luo M, Abbott D, Filali M, Chen N, Sigmund CD and Engelhardt JF.

(2004). Characterization of Lef-1 Promoter Segments that Facilitates Inductive Developmental Expression in Skin. JID, 123:2, 264-274. (Cover Image) PMCID: 1803077

114. Driskell RR, Liu X, Luo M, Filali M, Abbott D, Chen N, Moothart C, Sigmund CD and

Engelhardt JF. (2004). Wnt-Responsive Element Controls Lef-1 Promoter Expression During Submucosal Gland Morphogenesis. Am J Physiol Lung Cell Mol Physiol 287:L752-L763.

115. Zimmerman MC, Dunlay RP, Lazartigues E, Zhang Y, Sharma RV, Engelhardt JF,

Davisson, R.L. (2004). Requirement for Rac1-dependent NADPH oxidase in the cardiovascular and dipsogenic actions of angiotensin II in the brain. Circ. Res., 95, 532-539.

116. Zhang LN, Karp

P, Gerard

CJ, Pastor

E, Munson

K, Yan

Z, Godwin

S, Thomas

C, Zabner

, J,

Peluso R, Carter

B, and Engelhardt

JF. (2004). Dual Therapeutic Utility of Proteasome

Modulating Agents for Pharmico-Gene Therapy of the Cystic Fibrosis Airway. Molecular Therapy 10:6, 990-1002.

117. Terui K, Enosawa S, Haga S, Zhang HQ, Kuroda H, Kouchi K, Matsunaga T, Yoshida H,

Engelhardt JF, Irani K, Ohnuma N, and Ozaki M. (2004) Stat3 confers resistance against hypoxia/reoxygenation-induced oxidative injury in hepatocytes through upregulation of Mn-SOD. J Hepatolgy 41 957-965.

118. Yan Z., Zak R, Zhang Y, and Engelhardt JF. (2005). Inverted terminal repeat sequences are

important for intermolecular recombination and circularization of adeno-associated virus genomes. J Virology 79:1, 364-379. PMCID: 538689

119. Panopoulos A. Harraz M, Engelhardt JF and Zandi E (2005). Iron-mediated H2O2

production as a mechanism for cell type-specific inhibition of tumor necrosis factor alpha-induced but not interleukin-1beta-induced IkappaB kinase complex/nuclear factor-kappaB activation. J. Biol. Chem 280: 2912-2923.

http://www.rbej.com/content/1/1/83

http://www.rbej.com/content/1/1/78


120. Dajani R, Zhang Y, Taft PJ, Travis SM, Starner TD, Olsen A, Zabner J, Welsh, MJ and Engelhardt JF (2005). Lysozyme Secretion by Submucosal Glands Protects the Airway from Bacterial Infection. Am J Respir Cell Mol Biol, 32, 548-552. PMCID: 2715323

121. Ding W, Zhang L, Yan Z, and Engelhardt JF. (2005) Intracellular trafficking of adeno-

associated viral vectors. Gene Therapy, 12, 873-880. 122. Liu X, Luo M, Zhang LN, Yan Z, Zak R, Ding W, Mansfield SG, Mitchell LG and Engelhardt

JF (2005). Spliceosome-Mediated RNA Trans-Splicing with Recombinant Adeno-Associated Virus Partially Restores Cystic Fibrosis Transmembrane Conductance Regulator Function to Polarized Human Cystic Fibrosis Airway Epithelial Cells. Human Gene Therapy, 16,1116-1123.

123. Li Z, Sun X, Chen J, Leno GH, and Engelhardt JF. (2005). Factors Affecting the

Electrofusion of Mouse and Ferret Oocytes With Ferret Somatic Cells. Mol Rep Dev 72, 40-47.

124. Li Z, Chen J, Sun X, Zhou Q, Chen J, Leno GH and Engelhardt JF. (2005). Nuclear Transfer

of M-phase Ferret Fibroblasts Synchronized with the Microtubule Inhibitor Demecolcine. J Exp Zool 303A:1126-1134. http://www.ncbi.nlm.nih.gov/pubmed/16254916?dopt=Citation

125. Lai Y, Yue Y, Liu M, Ghosh A, Engelhardt JF, Chamberlain JS and Duan D. (2005). Efficient

in vivo gene expression by trans-splicing adeno-associated viral vectors. Nature Biotech, 23, 1435-1439. PMCID: 2581721

126. Vadlamudi U, Espinoza HM, Ganga M, Martin DM, Liu X, Engelhardt JF, and Amendt BA.

(2005) PITX2, Beta-catenin, and LEF-1 Interact to Synergistically Regulate the LEF-1 Promoter. J. Cell Sci., 118, 1129-1137. http://www.ncbi.nlm.nih.gov/pubmed/15728254?dopt=Citation

127. Li Q, Harraz MM, Zhou W, Zhang L, Ding W, Zhang Y, Eggleston T, Yeaman C, Banfi B, and

Engelhardt JF. (2006). Nox2 and Rac1 Regulate H2O2-Dependent Recruitment of TRAF6 to Endosomal Interleukin-1 Receptor Complexes. Mol Cell Bio, 26, 140-154. PMCID: 1317618

128. Liu X, Yan Z, Luo M, and Engelhardt JF (2006) Species-Specific Differences in Mouse and

Human Airway Epithelial Biology of Recombinant Adeno-Associated Virus Transduction. Am J Respir Cell Mol Biol.34, 56-64 (Cover Image). PMCID: 1752084

129. Li Q, and Engelhardt, JF. (2006). Interleukin-1beta Induction of NFkappaB is Partially

Regulated by H2O2-mediated Activation of NFkappaB-inducing Kinase. J Bio Chem, 281:3, 1495-1505.

130. Li Z, Sun X, Chen J, Liu X, Wisely SM, Zhou Q, Renard J-P, Leno GH and Engelhardt JF.

(2006). Cloned Ferrets Produced by Somatic Cell Nuclear Transfer. Develop Bio, 293, 439-448. PMCID: 1892907

131. Ding W, Zhang LN, Yeaman C, and Engelhardt JF (2006). rAAV2 Traffics through both the

Late and the Recycling Endosomes in a Dose-Dependent Fashion. Molecular Therapy, 13(4), 671-682. PMCID: 1712670

132. Li, Z., Sun, X., Chen, J., Leno, G.H. and Engelhardt, J.F. (2006). Factors affecting the

efficiency of embryo transfer in the domestic ferret (Mustela putorius furo). Theriogenology, 66(2)183-90. PMCID: 1893552

133. Hingtgen, S.D., Yang, J., Dunlay, S.M., Sharma, R.V., Engelhardt, J.F., and Davisson, R.L.

(2006). Nox2-containing NADPH oxidase and AKt activation play a key role in angiotensin II-induced cardiomyocyte hypertrophy. Physiological Genomics, 26, 180-191.

http://www.ncbi.nlm.nih.gov/pubmed/16254916?dopt=Citation



134. Yan, Z., Lei-Butters, D.C.M., Liu, X., Zhang, Y., Zhang, L., Luo, M., Zak, R. and Engelhardt, J.F. (2006). Unique Biological Properties of Recombinant AAV1 Transduction in Polarized Human Airway Epithelia. J Biolog Chem., 281(40), 29684-29692. PMCID: 1712671

135. Engelhardt, J.F. (2006). AAV Hits the Genomic Bull’s-Eye: Adeno-associated Virus Mediates

Correction of Genetic Mutations in Mouse Liver. Nature Biotech, 24(8), 949-950. Publication was accepted for publication before April 7, 2008

136. Liu, X., Driskell, R.R., and Engelhardt, J.F. (2006). Stem Cells in the Lung. Methods

Enzymol, 419: 285-321. PMCID: 1803078 137. Yan, Z., Lei-Butters, D.C.M., Zhang, Y., Zak, R., and Engelhardt, J.F. (2007). Hybrid Adeno-

Associated Virus Bearing Nonhomologous Inverted Terminal Repeats Enhances Dual-Vector Reconstruction of Minigenes In vivo. Human Gene Therapy, 18: 81-87. PMCID: 2121583

138. Dajani, R., Sanlioglu, S., Zhang, Y., Li, Q., Monick, M.M., Lazartigues, E., Eggleston, T.,

Davisson, R.L., Hunninghake, G.H. and Engelhardt, J.F. (2007). Pleiotropic Functions of TNF{alpha} Determine Distinct IKK{beta}-Dependent Hepatocellular Fates in Response to LPS. Am J Physiol Gastrointest Liver Physiol. 292:G242-G252.

139. Liu, X., Luo, M., Zhang, L., Ding, W., Yan, Z and Engelhardt, J.F. (2007). Bioelectric

Properties of Chloride Channels in Human, Pig, Ferret, and Mouse Airway Epithelia. Am J Resp Cell Mol Biol 36(3), 313-323. (Cover Image). PMCID: 1894945

140. Driskell, R.R., Goodheart, M., Neff, T., Liu, X., Luo, M., Moothart, C., Sigmund, C.D.,

Hosokawa, R., Chai, Y., Engelhardt, J.F. (2007). Wnt3a Regulates Lef-1 Expression During Airway Submucosal Glad Morphogenesis. Developmental Biology, 305, 90-102. PMCID: 1892170

141. Fox, L.E., Green, D., Yan, Z., Engelhardt, J.F., and Wu, C.F. (2007). Screen for dominant

behavioral mutations caused by genomic insertion of p-element transposons in Drosophila: An examination of the integration of viral vector sequences. J Neurogenetics 21(1): 31-43. PMCID: 2121585

142. Nozoe, M., Hirooka, Y., Koga, Y., Sagara, Y., Kishi, T., Engelhardt, J.F., and Sunagawa, K.

(2007). Inhibition of Rac1-Derived Reactive Oxygen Species in Nucleus Tractus Solitarius Decreases Blood Pressure and Heart Rate in Stroke-Prone Spontaneously. Hypertension 50(1), 62-68. Publication was accepted for publication before April 7, 2008

143. Amen, M., Liu, X., Vadlamudi, U., Elizondo, G., Diamond, E., Engelhardt, J.F., and Amendt,

B.A. (2007). PITX2 and -catenin Interactions Regulate Lef-1 Isoform Expression. Molecular Cell Biology 27(21), 7560-7573. PMCID: 2169058

144. Marden, J.J., Harraz, M.M., Williams, A.J., Nelson, K., Luo, M., Paulson, H., and Engelhardt,

J.F. (2007). Redox Modifier Genes in Amyotrophic Lateral Sclerosis. J Clinical Invest. 17:2913-2919. PMCID: 1974865

145. Liu, X., Luo, M., Guo, C., Yan, Z., Wang, Y., and Engelhardt, J.F. (2007). Comparative

Biology of rAAV Transduction in Ferret, Pig and Human Airway Epithelia. Gene Therapy, 21. 1543-1548. PMCID: 2121584

146. Liu, X., Luo, M., Trygg, C., Yan, Z., Lei-Butters, D.C.M., Smith, C.B., Fischer, A.C., Munson,

K., Guggino, W.B., Bunnell, B.A. and Engelhardt, J.F. (2007). Biological Differences in rAAV Transduction of Human and Old World non-Human Primate Airway Epithelia. Mol Therapy 15(21), 2114-2123. PMCID: 2121582


147. Harraz, M.M., Park, A., Abbott, D., Zhou, W., Zhang, Y. and Engelhardt, J.F. (2007). MKK6 Phosphorylation Regulates Production of Superoxide by Enhancing Rac GTPase Activity. Antioxidants Redox Signal. 9(11), 1803-1813. PMCID: 3597079

148. Harraz, M.M., Marden, J.J., Zhou, W., Zhang, Y., Williams, A., Sharov, V.S., Nelson, K., Luo,

M., Paulson, H., Schoneich, C., and Engelhardt, J.F. (2008). SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model. J Clin Invest 118: 659-670. PMCID: 2213375

149. Crystal, R.G., Randell, S.H., Engelhardt, J. F., Voynow, J., and Sunday, M.E. (2008). Airway

epithelial cells: current concepts and challenge. Proc. Am Thorac. Soc., 5(7), 772-777. Publication was accepted for publication before April 7, 2008

150. Franks, T.J., Colby, T.V., Travis, W.D., Tuder, R.M., Reynolds, H.Y., Brody, A.R., Cardoso,

W.V., Crystal, R.G., Drake, C.J., Engelhardt, J., Frid, M., Herzog, E., Mason, R., Phan, S.H., Randell, S.H., Rose, M.C., Stevens, T., Serge, J., Sunday, M.E., Voynow, J.A., Weinstein, B.M., Whitsett, J. and Williams, M.C. (2008). Resident cellular components of the human lung: current knowledge and goals for research on cell phenotyping and function.Proc. Am Thorac. Soc., 5(7), 763-766. Publication was accepted for publication before April 7, 2008

151. Li, Q., Zhang, Y.T., Marden, J.J., Banfi, B., and Engelhardt, J.F. (2008). Endosomal NADPH

oxidase regulates c-Src activation following hypoxia/reoxygenation injury. Biochem J. 411:3, 531-541. PMCID: 3597079

152. Marden, J.J., Zhang, Y., Oakley, F.D., Zhou, W., Luo, M., Jia, H.P., McCray, P.B., Jr., Yaniv,

M., Weitzman, J.B., and Engelhardt J.F. (2008). JunD protects the liver from ischemia/reperfusion injury by dampening AP-1 Transcriptional activation. J Biol Chem. 283(11), 6687-6695. PMCID: 3593645

153. Sun, X., Yan, Z., Yi, Y., Li, Z., Lei, D., Rogers, C.S., Chen, J., Zhang, Y., Welsh, M., Leno,

G.H. Engelhardt, J.F. (2008) AAV Targeted Disruption of the CFTR Gene in Cloned Ferrets. J. Clinic Invest 118:4, 1578-1583. PMCID: 2263147

154. Rogers, C.S., Hao, Y., Rokhlina, T., Samuel, M., Stoltz, D.A., Li, Y., Petroff, E., Vermeer,

D.W., Kabel, A.C., Yan, Z., Spate, L., Wax, D., Murphy, C.N., Rieke, A., Whitworth, K., Linville, M.L., Korte, S.W., Engelhardt, J.F., Welsh, M.J., Prather, R.S. (2008). Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer, J Clin Invest 118:4, 1571-1577. PMCID: 2265103

155. Peterson, J.R., Infanger, D.W., Bragga, V.A., Zhang, Y., Sharma, R.V., Engelhardt, J.F., and

Davisson, R.L. (2008). Longitudinal non-invasive Monitoring of Transcription Factor Activation in Cardiovascular Regulatory Nuclei Using Bioluminescence Imaging. Physiol Genomics, 33(2), 292-299. Publication was accepted for publication before April 7, 2008

156. Mumbengegwi, D.R., Li, Q., Li, C., Bear, C.E., and Engelhardt, J.F. (2008). Evidence for a

Superoxide Permeability Pathway in Endosomal Membranes. Mol Cell Biol. 28(11): 3700-3712. PMCID: 2423302.

157. Rogers, C.S., Abraham, W.M., Brogden, K.A., Engelhardt, J.F., Fisher, J.T., McCray, P.B.,

Jr., McLennan, G., Meyerholz, D.K., Namati, E., Ostedgaard, L.S., Prather, R.S., Sabater, J.R., Stoltz, D.A.,. Zabner, J., Welsh, M.J. (2008). The porcine lung as a potential model for cystic fibrosis, Am J Physiol Lung Cell Mol Physiol, 295(2), L240-63. PMCID: 2519845

158. Hsu, M.Y, Rovinsky, S.A, Lai, C.Y, Qasem, S., Liu, X., How, J., Engelhardt, J.F., Murphy,

G.F. (2008). Aggressive melanoma cells escape from BMP7-mediated autocrine growth inhibition through coordinated Noggin upregulation. Lab Investigation, Aug;88(8):842-55. PMCID: 2676927

http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=Search&Term=%22Hsu%20MY%22%5BAuthor%5D&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVCitation

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159. Liu, X., and Engelhardt, J.F. (2008). The glandular stem/progenitor cell niche in airway

development and repair. Proc. Am Thorac. Soc., 5(6), 682-688. PMCID: 2645260 160. Sun, X., Li, Z., Yi, Y., Chen, J., Leno, G.H., Engelhardt, J.F. (2008). Efficient Term

Development of Vitrified Ferret Embryos Using a Novel Pipette Chamber Technique. Biol Reprod. 79(5), 832-840. PMCID: 2658816

161. Liu, X., Luo, M., Guo, C., Yan, Z., Wang, Y., Lei-Butters, D.C. and Engelhardt, J.F. (2009).

Analysis of Adeno-Associated Virus Progenitor Cell Transduction in Mouse Lung. Molecular Therapy, 17(2), 285-293. PMCID: 2665184

162. Sun, X., Li, Z., Yi, Y., Ding, W., Chen, J., Engelhardt, J.F., and Leno, G.H. (2009).

Chromatin Configurations in the Ferret Germinal Vesicle that Reflect Developmental Competence for In Vitro Maturation. Reprod Domest Anim. 44,320-325. http://www.ncbi.nlm.nih.gov/pubmed/18992097?dopt=Citation PMCID: 3621713

163. Yan, Z., Sun, X., and Engelhardt, J.F. (2009). Progress and prospects: techniques for site-

directed mutagenesis in animal models. Gene Therapy, 16, 581-588. PMCID: 2798581 164. Li, Q., Spencer, N.Y., Oakley, F.D., Buettner, G.R., and Engelhardt, J.F. (2009). Endosomal

Nox2 Facilitates Redox-Dependent Induction of NFkappaB by TNFalpha. Antioxidants and Redox Signaling 11(6), 1249-1263. PMCID: 2842115

165. Oakley, F.D., Abbott, D., Li, Q., and Engelhardt, J. (2009). Signaling Components of Redox

Active Endosomes: The Redoxosomes. Antioxid Redox Signal. 11(6), 1313-1333. PMCID: 2842130

166. Carter, B.J., Anklesaria, P., Choi, S., and Engelhardt, J.F. (2009). Redox modifier genes

and pathways in amyotrophic lateral sclerosis. Antioxid Redox Signal., 11(7), 1569-1586. PMCID: 2842588

167. Yan, Z., Lei-Butters, D., Engelhardt, J.F. and Leno, G.H. (2009). Indexing TNF-alpha gene

expression using a gene-targeted reporter cell line. BMC Biol, 7:8. PMCID: 2657777 168. Oakley, F.D., Smith, R.L., and Engelhardt, J. (2009) Lipid Rafts and Caveolin-1 Coordinate

IL-1-Dependent Activation of NFB by Controlling Endocytosis of Nox2 and IL-1R1 from the Plasma Membrane. J Biol Chem. 284:48, 33255–33264. PMCID: 2785168

169. Xu, G., Zhang, Y., Jia, H., Li, J., Liu, X., Engelhardt, J.F., and Wang, Y. (2009). Cloning and

identification of microRNAs in bovine alveolar macrophages. Mol Cell Biochem 332:9-16. Publication was not directly supported by NIH.

170. Sisson, T.H., Mendez, M., Choi, K., Subbotina, N., Courey, A., Cunningham, A., Dave, A.,

Engelhardt, J.F., Liu, X., White, E.S., Thannicka, V.J., Moore, B.B., Christensen, P.J. and Simon, R.H. (2010). Targeted Injury of Type II Alveolar Epithelial Cells Induces Pulmonary Fibrosis. Am J Respir Crit Care Med., 181, 254-263. PMCID: 2817814

171. Flotte, T.R., Fischer, A.C., Goetzmann, J., Mueller, C., Cebotaru, L., Yan, Z., Wang, L.,

Wilson, J.M., Guggino, W.B., and Engelhardt, J.F. (2010). Dual Reporter Comparative Indexing of rAAV Pseudotyped Vectors in Chimpanzee Airway. Mol Ther. 18(3), 594-600. PMCID: 2839428

172. Sun, X., Sui, H., Fisher, J.T., Yan, Z., Liu, X., Cho, H-J., Joo, N.S., Zhang,Y., Zhou,W., Yi,Y.,

Kinyon, J.M., Lei-Butters, D.C., Griffin, M.A., Naumann, P., Luo, M., Ascher, J., Wang, K., Frana, T., Wine, J.J., Meyerholz, D.K., and Engelhardt, J.F. (2010). Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. J. Clinical Invest. 120(9), 3149-3160. PMCID: 2929732



173. Liu, X., Luo, M., Xie, W., Wells

, J.M., Goodheart

, M.J., and Engelhardt

, J.F. (2010). Sox17

Modulates Wnt3A/-catenin-Mediated Transcriptional Activation of the Lef-1 Promoter. Am. J. Physiol Lung Cell Mol Physiol. 299:L694-L710. PMCID: 2980392

174. Spencer, NY, Yan, Z, Boudreau, RL, Zhang, Y, Luo, M., Li, Q., Tian, X., Shah, A.M.,

Davisson, R.L., Davidson, B., Banfi, B. and Engelhardt, J.F. (2011). Control of hepatic nuclear superoxide production by glucose 6-phosphate dehydrogenase and NADPH oxidase-4, The Journal of Biological Chemistry, 286(11), 8977-8987. PMCID:3059029

175. Zhang, Z. & Liu, X. Wu, T. Liu, J., Zhang, X., Yang, X., Goodheart, M.J., Engelhardt, J.F.

and Wang, Y. (2011). Selective suppression of cervical cancer Hela cells by 2-O-β-D-glucopyranosyl-L-ascorbic acid isolated from the fruit of Lycium barbarum L. Cell Biol Toxicol. 27:107–121. Publication was not directly supported by NIH.

176. Xie, W., Fisher, J.T., Lynch, T.J., Luo, M., Evans, T.I.A., Neff, T.L., Zhou, W., Zhang, Y., Ou,

Y., Bunnett, N.W., Russo, A.F., Goodheart, M.J., Parekh, K.R., Liu, X., and Engelhardt, J.F. (2011). CGRP Induction in cystic fibrosis airways alters the submucosal gland progenitor cell niche in mice. J Clin Invest. 121(8): 3144-3158. PMCID:3148720

177. Li, Y., Wang, J., He, H.Y., Ma, L.J., Zeng, J., Deng, G.C., Liu, X, Engelhardt, J.F., Wang, Y.

(2011). Immunohistochemical demonstration of airway epithelial cell markers of Guinea pig. Tissue and Cell 43:283-290. PMCID: 3622455

178. Keiser, N.W. and Engelhardt, J.F. (2011). New animal models of cystic fibrosis: what are

they teaching us? Curr Opin Pulm Med.17: 478-483. PMCID: 3596000 179. Keiser, N.W., Yan, Z., Zhang, Y., Lei-Butters, D.C.M. and Engelhardt, J.F. (2011). Unique

characteristics of AAV1, 2, and 5 Viral Entry, Intracellular Trafficking, and Nuclear Import Define Transduction Efficiency in HeLa Cells. Human Gene Therapy, 22(11), 1433-1444. PMCID: 3225038

180. Li, Q., Spencer, N.Y., Pantazis, N.J. and Engelhardt, J.F. (2011). ALSIN and SOD1G93A

regulate endosomal ROS production by glial cells and pro-inflammatory pathways responsible for neurotoxicity. J. Biol. Chem. 26(24), 40151-40162. PMCID: 3220533

181. Fisher, J.T., Zhang, Y., and Engelhardt, J.F. (2011). Comparative biology of cystic fibrosis

animal models. Methods Mol Biol 742: 311-334. 182. Ramsey, B.W., Banks-Schlegel, S., Accurso, F.J., Boucher, R.C., Cutting, G.R., Engelhardt,

J.F., Guggino, W.B., Karp, C.L., Knowles, M.R., Kolls, J.K., LiPuma, J.J., Lynch, S., McCray, P.B. Jr., Rubenstein, R.C., Singh, P.K., Sorscher, E. and Welsh, M. (2012). Future Directions in Early Cystic Fibrosis Lung Disease Research. Am J Resp Critic Care Med. 185(8), 887-892. PMCID: 3360572.

183. Shelton, D. N., Fornalik, H., Neff, T., Park, S. Y., Bender, D., Degeest, K., Liu, X., Xie, W.,

Meyerholz, D. K., Engelhardt, J. F., Goodheart, M. J. (2012). The Role of LEF1 in Endometrial Gland Formation and Carcinogenesis. PLoS One 7(7), 40312. PMCID: 3391280.

184. Kaminsky, P.M., Keiser, N.W. Yan, Z., Lei-Butters, D.C.M., and Engelhardt, J.F. (2012).

Directing Inegrin-linked Endocytosis of Recombinant AAV enhances Productive FAK-dependent transduction. Molecular Therapy 20(5), 972-983. PMCID: 3345987

185. Fisher, J.T., Liu, X., Yan, Z., Luo, M., Zhang, Y., Zhou, W., Lee, B.J., Song, Y., Guo, C.,

Wang, Y., Lukacs, G.L. and Engelhardt, J.F. (2012). Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator. The Journal of Biological Chemistry 287(26), 21673-21685. PMCID: 3381131


186. Olivier, A.K., Yi, Y., Sun, X., Sui, H., Liang, B., Hu, S., Xie, W., Fisher, J.T., Keiser, N.W., Lei-Butters, D.C., Zhou, W., Yan, Z., Li, G., Evans, T.I.A., Meyerholz, D.K., Wang, K., Stewart, Z.A., Norris, A.W. and Engelhardt, J.F. (2012). Abnormal Endocrine Pancreas Function at Birth in Cystic Fibrosis Ferrets. Journal of Clinical Investigation 122(10):3755–3768. PMCID: 3534166

187. Huang, Q., Deng, X., Yan, Z., Cheng, F., Luo, Y., Shen, W., Lei-Butters, D.C.M., Chen, A.Y.,

Li, Y., Tang, L., Soderlund-Venermo, M., Engelhardt, J.F., and Qiu, J. (2012). Establishment of a Reverse Genetics System for the Study of Human Bocavirus and Evidence of Cytopathogenesis in the Polarized Human Airway Epithelium. PLoS Pathogens 8(8), e1002899. PMCID: 3431310

188. Nakano, Y., Jahan, I., Bonde, G., Sun, X., Hildebrand, M.S., Engelhardt, J.F., Smith, R.J.H.,

Cornell, R.A., Fritzsch, B., and Bánfi, B. (2012). A Mutation in the Srrm4 Gene Causes Alternative Splicing Defects and Deafness in the Bronx Waltzer Mouse. PloS Genetics, 8(10), e1002966. PMCID: 3464207

189. Yan, Z., Lei-Butters, D.C., Keiser, N.W., and Engelhardt, J.F. (2013). Distinct transduction

difference between adeno-associated virus type 1 and type 6 vectors in human polarized airway epithelia. Gene Therapy 20(3), 328-37. PMCID: 3443503

190. Sui H, Olivier AK, Klesney-Tait JA, Brooks L, Tyler SR, Sun X, Skopec A, Kline J, Sanchez

PG, Meyerholz DK, Zavazava N, Iannettoni M, Engelhardt JF, Parekh KR. (2013). Ferret Lung Transplant: An Orthotopic Model of Obliterative Bronchiolitis. Am J. Transplant 13(2), 467-476. PMCID: 3638989

191. Deng, X., Yan, Z., Luo, Y., Xu, J., Cheng, F. Li, Y., Engelhardt, J.F., and Qiu, J. (2013). In

vitro modeling of human bocavirus 1 infection of polarized primary human airway epithelia. Journal of Virology 87(7):4097-102. PMCID: 3624236

192. Yan, Z., Keiser, N.W., Song, Y., Deng, X., Cheng, F., Qiu, J., and Engelhardt, J.F. (2013). A

novel chimeric adeno-associated virus 2 / human bocavirus 1 parvovirus vector efficiently transduces human airway epithelia. Molecular Therapy 20(3), 328-337. PMCID: 3443503

193. Keiser, N.W. and Engelhardt, J.F. (2013). Gene delivery to the airway. Curr Protoc Hum

Genet, Chapter 13. (Unit13 9). Lung phon 194. Spencer, N.Y., Zhou, W., Li, Q., Zhang, Y., Luo, M., Yan, Z., Lynch, T.J., Abbott, D., Banfi,

B., and Engelhardt, J.F. (2013). Hepatocytes Produce TNFalpha Following Hypoxia/Reoxygenation and Liver Ischemia/Reperfusion in a NADPH Oxidase- and c-Src-Dependent Manner. Am J Physiol Gastrointest Liver Physiol 305(1), G84-94. PMCID: 3725690

195. Yan, Z., Sun, X., Evans, I.A., Tyler, S.R., Song, Y., Liu, X., Sui, H., and Engelhardt, J.F. (2013) Postentry processing of recombinant adeno-associated virus type 1 and transduction of the ferret lung are altered by a factor in airway secretions. Human Gene Therapy 24(9), 786-796. PMCID: 3768237

196. Fisher, J. T., Tyler, S. R., Zhang, Y., Lee, B. J., Liu, X., Sun, X., Sui, H., Liang, B., Luo, M.,

Xie, W., Yi, Y., Zhou, W., Song, Y., Keiser, N., Wang, K., de Jonge, H. R., and Engelhardt, J. F. (2013) Bioelectric Characterization of Epithelia from Neonatal CFTR Knockout Ferrets. Am J Respir Cell Mol Biol 49(5), 837-844. PMCID: 3931095

197. Sun, X., Olivier, A.K., Liang, B., Yi, Y., Sui, H., Evans, T.I.A., Zhang, Y., Zhou, W., Tyler,

S.R., Fisher, J.T., Keiser, N.W., Liu, X., Yan, Z., Song, Y., Goeken, J.A., Kinyon, J.M., Fligg, D., Wang, X., Xie, W., Lynch, T.J., Kaminsky, P.M., Stewart, Z.A., Pope, R.M., Frana, T., Meyerholz, D.K., Parekh, K., and Engelhardt, J.F. 2014. Lung Phenotype of Juvenile and Adult CFTR-Knockout Ferrets Am J Respir Cell Mol Biol ;50(3):502-12.

http://www.ncbi.nlm.nih.gov/pubmed?term=Sui%20H%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Olivier%20AK%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Klesney-Tait%20JA%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Brooks%20L%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Tyler%20SR%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Sun%20X%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Skopec%20A%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Kline%20J%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Sanchez%20PG%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Sanchez%20PG%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Meyerholz%20DK%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Zavazava%20N%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Iannettoni%20M%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Engelhardt%20JF%5BAuthor%5D&cauthor=true&cauthor_uid=23205765

http://www.ncbi.nlm.nih.gov/pubmed?term=Parekh%20KR%5BAuthor%5D&cauthor=true&cauthor_uid=23205765


198. Peng, X., Gori, K., Alfoldi, J., Eisfeld, A.J., Tyler, S.R., Tisoncik-Go, J., Brawand, D., Law, L.,

Skunca, N., Hatta, M., Gasper, D.J., Kelly, S.M. Chang, J., Thomas, M., Johnson, J., Berlin, A.M., Lara, M., Russell, P., Swofford, R., Turner-Maier, J., Young, S., Hourlier

T., Aken, B.,

Searle, S., Sun, S., Yi, Y., Suresh, M., Tumpey, T.M., Siepel, A., Wisely, S.M., Dessimoz, C., Kawaoka, Y., Birren, B.W., Lindblad-Toh, K., Di Palma, F., Engelhardt, J.F., Katze, M.G., and Palermo, R.E. 2013. Insights from the ferret genome (M. putorius furo) for biomedical models of human respiratory disease. Nature Biotechnology. (Submitted).

199. Sun, X., Olivier, A.K., Yi, Y., Pope, C.E., Hayden, H.S., Liang, B., Sui, H., Zhou, W., Hager,

K.R., Zhang, Y., Liu, X., Yan, Z., Fisher, J.T., Keiser, N.W., Song, Y., Tyler, S.R., Goeken, J.A., Kinyon, J.M., Radey, M.C., Fligg, D., Wang, X., Xie, W., Lynch, T.J., Kaminsky, P.M., Brittnacher, M.J., Miller, S.I., Parekh, K., Meyerholz, D.K., Hoffman, L.R., Frana, T., Stewart, Z.A., and Engelhardt, J.F. 2014. Gastrointestinal Pathology in Juvenile and Adult CFTR-Knockout Ferrets. American Journal of Pathology (In press).

200. Spencer, N.Y. and Engelhardt, J.F. 2014. The basic biology of redoxosomes in cytokine-mediated signal transduction and implications for disease-specific therapies. Biochemistry; 53(10):1551-64. PMCID: 3985689.

Non-Peer Reviewed: Journals, Reviews, and Book Chapters

1. Engelhardt JF and Wilson JM. Gene therapy of cystic fibrosis lung disease. J Pharm

Pharmacol Suppl 1:165-167, (1992). 2. Engelhardt JF. "Epithelial Cell Biology Relevant to Gene Therapy of Cystic Fibrosis" in

Highlights review of North American Cystic Fibrosis Conference meeting, SynerMed, Inc (1995).

3. Engelhardt JF. "Methods of Adenoviral-Mediated Gene Transfer to Airway Epithelium" in

Methods in Molecular Biology: Gene Therapy Protocols, Humana Press, Inc. (1996). 4. Engelhardt JF and Wilson JM. "Explant Models of the Airway" in The Lung, Raven Press,

Inc. ed Crystal and West, 23:345-352 (1997). 5. Jiang Q and Engelhardt JF. "Cellular Heterogeneity of CFTR Expression and Function in the

Lung: Implications for Gene Therapy of Cystic Fibrosis" European Journal of Human Genetics 6:12-31 (1998).

6. Duan D, Zhang Y, and Engelhardt JF. “Gene Delivery to the Airway” in Current Protocols in

Human Genetics, John Wiley & Sons, Inc. 13:9.1-9.34 (1998). 7. Engelhardt, JF. “Redox Mediated Gene Therapies for Environmental Injury: Approaches and

Concepts” in Antioxidant and Redox Signaling 1:5-27 (1999). 8. Fan C, Zwacka RM, and Engelhardt JF. “Therapeutic Approaches for Ischemia/Reperfusion

Injury in the Liver” in Journal of Molecular Medicine 77:577-596 (1999). 9. Duan D, and Engelhardt JF. “Stretching it: Expanded Applications of Recombinant Adeno-

associated Viral Vectors for Gene Therapy” in Biotech Lab International 10-12 (March-April, 2001).

10. Engelhardt, J.F. Summary Report of a Symposium: Genes and Gene Delivery for Diseases of

Alcoholism. Alcoholism: Clinical and Experimental Research 25:12, 1778-1880 (2001).


11. Filali, M., Zhang, Y., Ritchie, T.C., and Engelhardt, J.F. Xenograft model of the CF airway. Methods Mol Med 70:537-550 (2002)

12. Yan, Z., Ritchie, T. C., Duan, D. and Engelhardt, J. F. Recombinant AAV-mediated gene

delivery using dual vector heterodimerization. Methods Enzymol 346, 334-5 (2002).

13. Filali M, Liu X, Cheng N, Abbot D, Leontiev V, and Engelhardt JF. Mechanisms of submucosal gland morphogenesis in the airway. Mucus Hypersecretion in Respiratory Disease. Wiley, Chichester (Novartis Foundation Symposium 248), 38-50 (2002).

14. Duan D, Yue Y and Engelhardt JF. "Adeno-Associated Virus" in Gene Therapy in Lung

Disease, Marcel Dekker, Inc. ed Albelda, 51-92 (2002).

15. Duan D, Yue Y, Yan Z and Engelhardt JF. Trans-Splicing Vectors Expand the Packaging Limits of Adeno-Associated Virus for Gene Therapy Applications. Methods Mol Med 76:287-307 (2002).

16. Duan D, Yue Y and Engelhardt JF. "Dual Vector Expansion of the Recombinant AAV

Packaging Capacity" in Methods in Molecular Biology Cardiac Cell and Gene Transfer by J.M. Metzger, Humana Press, Inc., Totowa NJ. 219:29-51 (2002).

17. Driskell, R.A., and Engelhardt, JF. "Current Status of Gene Therapy for Inherited Lung

Diseases" in Ann. Rev. Physiol. 65: 585-612 (2003). PMCID: 1857313

18. Yang J, Ritchie TC and Engelhardt JF. “Model System for Developing Gene Therapy Approaches for Myocardial Ischemia/Reperfusion Injury” in Methods in Enzymology 353: 321-336 (2003).

19. Engelhardt JF, Qiang L, and Ritchie T. "Gene Transfer Strategies and Therapeutic

Applications for Cardiovascular Ischemic Diseases" in Molecular Basis for Microcirculatory Disorders by GW Schmid-Schonbein and DN Granger, Springer-Verlag, France Paris 453-484 (2003).

20. Driskell RR and Engelhardt JF "Stem Cells in the Adult Lung" in The Handbook of Stem

Cells by RP Lanza, JP Gearhart, B Hogan, D Melton, R Pedersen, J Thomson, and M West, Academic Press, 547-554 (2004).

21. Liu X, Driskell RR

and Engelhardt

JF “Airway Glandular Development and Stem Cells” in

Current Topics in Developmental Biology, 64: 33-56 (2004).

22. Yan Z, Duan D, and Engelhardt JF. (2006). “Mechanism of Recombinant Adeno-Associated Virus Transduction” in Parvovirus Handbook (eds. M.E. Bloom, S.F. Cotmore, R.M. Linden, C.R. Parrish, and J.R. Kerr), pp. 511-524. Hodder Arnold, London.

23. Duan D, Yan Z, and Engelhardt

JF. (2006). “Expanding the Capacity of AAV Vectors” in

Parvovirus Handbook (eds. M.E. Bloom, S.F. Cotmore, R.M. Linden, C.R. Parrish, and J.R. Kerr), pp. 525-532. Hodder Arnold, London.

24. Liu X, Driskell RR,

and Engelhardt JF: Stem Cells in the Lung (chapter 12). Methods in

Enzymology, Vol. 419: 285-321: (Stem Cells Volume 2: Adult Stem Cells, edited by Drs. Robert Lanza and Irina Klimanskaya). Elsevier/Academic Press, (2006).

25. Liu X, Engelhardt JF: The glandular stem cell niche in airway development and repair.

Proceedings of the American Thoracic Society, 5(6): 682-688 (2008). 26. Crystal, R.G., Randell, S.H., Engelhardt, J. F., Voynow, J., and Sunday, M.E. (2008). Airway

epithelial cells: current concepts and challenges. Proc Am Thorac Soc, 5(7), 772-777.


27. Franks, T.J., Colby, T.V., Travis, W.D., Tuder, R.M., Reynolds, H.Y., Brody, A.R., Cardoso, W.V., Crystal, R.G., Drake, C.J., Engelhardt, J., Frid, M., Herzog, E., Mason, R., Phan, S.H., Randell, S.H., Rose, M.C., Stevens, T., Serge, J., Sunday, M.E., Voynow, J.A., Weinstein, B.M., Whitsett, J. and Williams, M.C. (2008). Resident cellular components of the human lung: current knowledge and goals for research on cell phenotyping and function. Proc Am Thorac Soc, 5(7), 763-766.

28. Fisher, J., Zhang, Y., Engelhardt, JF. (2011) “Comparative biology of cystic fibrosis animal

models” in Methods in Molecular Medicine (edited by M.D. Amaral and K. Kunzelmann) . pp. 311-314. Springer Science and Business Media.

29. Sun, X., Yan, Z., Liu, X., Olivier, A. K. and Engelhardt, J. F. (2014) Genetic Engineering in the Ferret, in Biology and Diseases of the Ferret, 3 (eds J. G. Fox and R. P. Marini), John Wiley & Sons, Inc., Ames, USA. pgs. 665-683.

Lectures by Invitation and Chaired Sessions

4/92 "Animal Models of Gene Therapy for Cystic Fibrosis" - American Society for Clinical Pathologist, Boston, Massachusetts.

6/92 "Bronchial Xenograft Model of Cystic Fibrosis" - Cystic Fibrosis Conference, Kingsmill,

VA 10/92 "Xenograft model of CF/Models systems for the study of cystic fibrosis" - North American

Cystic Fibrosis Foundation Conference, Washington, DC. 1/93 "Bronchial Xenograft Model of Cystic Fibrosis" - Cystic Fibrosis Gene Therapy

Conference, Florida 6/93 "Amphibian Skin as a Model for Mucous Secretion" - Cystic Fibrosis Conference

Kingsmill, VA. 6/93 "Second Generation Adenovirus' for Gene Therapy" - Cystic Fibrosis Conference

Kingsmill, VA. 6/93 "Human Bronchial Xenograft Model of Cystic Fibrosis" - University of Pittsburgh,

Pittsburgh, Pennsylvania 10/93 "Submucosal glands-Model systems" - North American Cystic Fibrosis Foundation

Conference, Dallas, Texas 10/93 "Localization of CFTR" Chaired Session - North American Cystic Fibrosis Foundation

Conference, Dallas, Texas. 10/93 "Gene Therapy: Strategies for Cystic Fibrosis" Hahnemann University, Philadelphia

Pennsylvania 11/93 "Gene Therapy for Cystic Fibrosis Lung and Liver Disease: Model Systems" Hospital Ste.

Justine, Montreal, Canada 1/94 "Gene Therapy of the Lung in Cystic Fibrosis" Keystone Stem Cell Meeting, Taos, NM 3/94 "Animal Models of Cystic Fibrosis Gene Therapy" - Delaware Valley College,

Doylestown, PA


3/94 "Development of More Efficacious Recombinant Adenoviral Vectors for Gene Therapy" - Cambridge Healthtech Institute on Recent Developments in the Diagnosis and Treatment of Cystic Fibrosis, Alexandria, VA.

4/94 "Pathophysiologic Mechanisms of Airway Disease in Cystic Fibrosis" Human Bronchial

Xenograft Model - Pulmonary Science Conference, Philadelphia PA. 4/94 "Models of Gene Therapy for Cystic Fibrosis - McGill University Montreal, Canada,

Department of Human Genetics. 4/94 "Gene Therapy for Cystic Fibrosis" - Cambridge Healthtech Institute on Gene Therapy,

New Technologies and Applications, Bethesda, MD. 6/94 "Preclinical Trials with Adenovirus in Non-Human Primates" Chaired Session, Gene

Therapy Conference, Cystic Fibrosis Foundation and the FDA, Kingsmill, VA. 6/94 "Second Generation Adenoviruses Defective in E2a" - Gene Therapy Conference, Cystic

Fibrosis Foundation and the FDA, Kingsmill, VA. 9/94 "Second Generation Adenoviral Vectors Defective in E2a: Applications in Gene Therapy"

- University of Pittsburgh School of Medicine, Department of Molecular Genetics and Biochemistry, Pittsburgh, PA.

9/94 "New Developments in Gene Therapy of Cystic Fibrosis Lung Disease" - Great Lakes Lung Conference, Ontario, Canada. 10/94 "Regulation of CFTR Expression"- North American CFF conference, Orlando, FL. Session Chair of Workshop. 11/94 "Gene Therapy and the Pancreas" - 6th Meeting of the International Association of

Pancreatology, Chicago, Illinois. 11/94 "Model Systems of Cystic Fibrosis Lung Disease: Toward Gene Therapy" -Wistar

Institute, Philadelphia, PA. 2/95 "From the Bench to Bedside: Progress Toward Gene Therapy of Cystic Fibrosis"-

Pennsylvania College of Osteopathic Medicine. Philadelphia, PA 5/95 "New Developments in Gene Therapy of Cystic Fibrosis Lung Disease"- Howard

University, Washington, DC. 5/95 "Recombinant Adenoviruses"- Gene Therapy and the Lung Graduate Course, ATS

International Conference, Seattle, Washington. 6/95 "Gene Therapy of Respiratory Mucus Defects in Cystic Fibrosis"- Cystic Fibrosis

Foundation Conference on Gene Therapy, Kingsmill, VA. 6/95 "Pathophysiologic mechanisms of bacterial colonization in the CF airway"-Cystic Fibrosis

Foundation Conference on CFTR, Kingsmill, VA. 6/95 "Genes and Vectors" - Gene Therapy Conference, Canadian Cystic Fibrosis Foundation,

Toronto. 9/95 "Advances Towards Gene Therapy of Cystic Fibrosis" R & D symposium for Life

Technologies Inc. Galthersburg, Maryland. 10/95 "Epithelial Cell Biology Pertinent to Gene Therapy" Chairman of Workshop, North

American Cystic Fibrosis Foundation, Dallas, Texas.


11/95 "Progenitor-Progeny Relationships in the Airway: Cellular Targets for Gene Therapy of

Cystic Fibrosis" University of Iowa, Iowa City, Iowa. 1/96 "Progenitor-Progeny Relationships in the Airway: Cellular Targets for Gene Therapy of

Cystic Fibrosis" Johns Hopkins University, Baltimore, Maryland. 2/96 "Progenitor-Progeny Relationships in the Airway: Cellular Targets for Gene Therapy of

Cystic Fibrosis" Genzyme Inc., Framingham, Massachusetts. 3/96 "Gene Therapy of Reactive Oxygen Species Damage: Applications in Cancer and Liver

Transplantation" Johns Hopkins University Cancer Center, Baltimore, Maryland. 4/96 "Progenitor Cells of the Proximal Airway: Targets for Gene Therapy in CF" FASEB

Experimental Biology Symposium, Washington, DC

5/96 "Vectors for Gene Therapy" Graduate Short Course at University of Nebraska, Omaha, Nebraska.

6/96 "Regulation of Fluid Transport in the Airway: Evidence for CFTR Regulated Amilorde

Insensitive Na Channel"- Animal Models Session-Cystic Fibrosis Foundation Conference, Kingsmill, VA.

6/96 "Species Specific Functions of CFTR?"-Pathophysiology Session-Cystic Fibrosis

Foundation Conference, Kingsmill, VA. 9/96 "Pathophysiologic Considerations in the Gene Therapy of Cystic Fibrosis and Surrogate

Endpoints for Efficacy"-University of Washington, Seattle, Washington 10/96 "Altered Fluid and Electrolyte Balance in the CF Airway: Effects on Bacterial Colonization.

CFF Conference on Antimicrobial Peptides and CF airway Infections. 10/96 "Epithelial cell biology pertinent to gene therapy" Chairman of Workshop, North American

Cystic Fibrosis Foundation, Dallas, Texas. 10/96 "Electrolyte and Fluid Transport" Co-Chairman of Workshop, North American Cystic

Fibrosis Foundation, Dallas, Texas. 11/96 "Cellular and Subcellular sites of CFTR Expression in the Lung: Pathophysiologic

Relevance in Gene Therapy for CF"- INSERM CF Conference, Savoy, France. 11/96 "Cellular and Subcellular sites of CFTR Expression in the Lung: Pathophysiologic

Relevance in Gene Therapy for CF" Dupont Research Center, Wilmington, DE 12/96 "Cellular and Subcellular sites of CFTR Expression in the Lung: Pathophysiologic

Relevance in Gene Therapy for CF" Johns Hopkins University, Baltimore, MD 1/96 "Gene Therapy of Reactive Oxygen Species Damage in Liver Ischemic Injury"

Environmental Medicine, University of Pennsylvania, Philadelphia, PA. 2/97 "Stem Cells in the Airway: Targets for in utero Gene Therapy in Cystic Fibrosis"

University of Iowa, HHMI, Iowa City, Iowa. 3/97 "Gene Therapy for Cystic Fibrosis Airways Disease: The moving Targets" Eastern

Society for Pediatric Research, 1997 Annual Conference, Plenary Talk, Ocean City, NJ. 5/97 “Animal Models for CF Airways Disease” CFF/FDA Workship on Surrogate Markers for

CF Gene Therapy, 1997 NIH, Washington, DC.


6/97 “Submucosal Glands” - workshop on regulation of electrolyte and fluid balance in the

airway - Cystic Fibrosis Foundation Conference, Kingsmill, VA. (Chaired Session) 10/97 “Surrogate Endpoints for Gene Therapy of CF Lung Disease,” North American EF

Conference, Nashville, Tennessee. 10/97 “Epithelial Cell Biology Pertinent to Gene Therapy,” Chairman of Workshop, North

American CF Conference, Nashville, Tennessee. 10/97 “CFTR, Ion Transport & Lung Defense,” Symposium Co-Chair, North American CF

Conference, Nashville, Tennessee. 3/98 “Xenograft Model of CF Airways Disease,” Broken Arrow Research Conference,

Canadian Cystic Fibrosis Foundation, Mississauga, Ontario. 4/98 “Tracheobranchial Submucosal Gland Development,” ATS International Conference,

Chicago, Illinois. 5/98 “Molecular Mechanisms of Recombinant Adeno-Associated Virus Transduction and

Persistence”, University of Alabama, Birmingham. 5/98 “CFTR Associated ATP Release is Controlled by a ‘Chloride Sensor’ within the Channel

Pore”, University of Alabama, Birmingham. 6/98 “CFTR Associated ATP Release is Controlled by a ‘Chloride Sensor’ within the Channel

Pore”, Cystic Fibrosis Foundation Conference, Kingsmill, VA. 6/98 “Circular Intermediates are Involved in rAAV Transduction”, Cystic Fibrosis Foundation

Conference, Kingsmill, VA. 10/98 “Stem Cells in the Airway”, Symposium at 12

th Annual NACFF Conference, Montreal,

Canada. 11/98 “Barriers to Gene Therapy with Recombinant Adeno-associated Viruses”, DERC

Symposium, University of Iowa. 3/99 “Mechanisms of rAAV Transduction” Case Western Reserve University, Ohio. 4/99 “Barriers to Gene Therapy with Recombinant Adeno-associated Viruses in the Airway”,

Targeted Genetics, Seattle, Washington. 4/99 “Gene Therapy with DNA viruses in the 21

st century: A walk Through the Past and Look

Toward the Future”, University of Oklahoma, Oklahoma City, Oklahoma. 5/99 “AAV Circular Intermediates”, NIH workshop on gene therapy, Bethesda, Maryland. 6/99 “Molecular Mechanisms of AAV Transduction” Cystic Fibrosis Foundation Conference,

Kingsmill, VA. 6/99 “Molecular Mechanisms of AAV Transduction” American Society of Gene Therapy Annual

Meeting, Plenary Session Talk, Seattle, Washington. 11/99 “Gene Therapy for Ischemia/Reperfusion Injury in the Liver Through Redox Modulation of

Signal Transduction Cascades“ AASLD Meeting, Dallas, Texas. 2/00 "Molecular Mechanisms of AAV Transduction" Targeted Genetics, Seattle, Washington


2/00 "From the Membrane to the Nucleus: Taking Recombinant Adeno-Associated Virus in Through the Back Door to Improve Gene Therapy to the Airway" Pulmonary Research Conference, Iowa City, Iowa.

3/00 "Gene Therapies for Environmental Injuries with DNA Viruses Through Redox

Modulation" University of Michigan, Ann Arbor, Michigan. 4/00 "Transduction Biology of Adeno-Associated Virus: Lessons for Gene Therapy" Molecular

Biology Seminar, Iowa City, Iowa. 4/00 "Redox Modulating Gene Therapies for Ischemia Reperfusion Injury in the Heart and

Liver" Louisiana State University Medical Center, Shreveport, Louisiana. 5/00 "Novel Gene Therapy Approaches with Recombinant Adeno-Associated Virus" University

of Pennsylvania, Institute for Human Gene Therapy, Philadelphia, Pennsylvania 5/00 "From the Membrane to the Nucleus: Insights into Molecular Mechanisms of

Recombinant AAV Transduction in the Airway", University of Nebraska, Omaha, Nebraska.

5/00 Chaired and Presented at "Animal Models: Pros & Cons of each" Cystic Fibrosis

Foundation Conference, Kingsmill, VA. 6/00 Plenary Talk, Third Annual Meeting of the American Society of Gene Therapy. "Molecular

Barriers to AAV transduction", Denver, Colorado. 7/00 "From the Membrane to the Nucleus: Innovative Strategies to Improve Adeno-Associated

Virus as a Gene Therapy Vector", State-of-the-Art Lecture, 19th Annual Meeting American

Society for Virology, Colorado State University, Fort Collins, Colorado. 11/00 Plenary Talk, 14

th Annual North American Cystic Fibrosis Foundation Conference.

“Opening Padlocks to CFTR Gene Therapy in the Lung: Are AAV Vectors the Key?” Baltimore, M.D.

01/01 "Redox Mediated Gene Therapies for Ischemic/Reperfusion Injury in the Heart and

Liver", Stanford University, Stanford, CA. 04/01 "Redox Mediated Gene Therapies for Ischemia/Reperfusion Injury". Experimental Biology

Meeting, Orlando, FL. 04/01 "Redox Modulating Gene Therapies for Environmental Injuries", University of Alabama,

Birmingham, AL. 04/01 "Mechanisms of rAAV-2 Endocytosis and Nuclear Trafficking", ASM Conference on Viral

Gene Vectors: Molecular Biology, Design and Application, Banff, Alberta Canada. 04/01 "Building Improved AAV Vector Systems through the Basic Biology of Recombinant AAV

Transduction", Avigen Inc., Alameda, CA. 05/01 "Subcellular Site of Superoxide Dismutase Expression Differentially Controls AP-1

Activity and Injury in the Liver Following Ischemia/Reperfusion", University of North Carolina, Chapel Hill, NC.

06/01 "AAV Vector Biology", American Society of Gene Therapy Meeting, Seattle, WA. 06/01 "Functional Correction of CFTR Channel Activity in Polarized Airway Models Using RNA

Trans-splicing", Williamsburg CFF Meeting, Kingsmill, Williamsburg, VA.


06/01 "Submucosal Gland Functions in the Airway", Kingsmill, Williamsburg CFF Meeting, Williamsburg, VA.

09/01 "Intracellular Tollgates for Adeno-Associated Virus Gene Delivery", Indiana University

School of Medicine, Indianapolis, IN. 11/01 "Redox Modulating Gene Therapies for Environmental Injuries: Insights into the Diverse

Regulatory Pathways Controlling NFkB Activation and Cell Survival", University of Southern California, Los Angeles, CA.

01/02 "Superoxides, the Good, the Bad, and the Ugly", American Society of Transplant

Surgeons Winter Symposium, Miami Beach, FL. 02/02 "Mechanisms of Submucosal Gland Morphogenesis in the Airway", Novartis Foundation

Symposium, London, United Kingdom. 03/02 "Diversity of Redox Activation Pathway for NFkB in Response to Environmental Injuries",

Oxygen Club of California, 2002 World Congress, Santa Barbara, CA. 04/02 "Airway stem cell targets for gene therapy of Cystic Fibrosis", Cystic Fibrosis and

Neuromuscular Diseases: Perspectives from Cell Therapy, Paris, France. 04/02 "Airway stem cell targets for gene therapy", Alpha-1 Foundation Critical Issues Workshop

Series No. 5, Miami, Florida. 06/02 "RNA-mediated Trans-splicing (SmaRT) Approaches for Gene Therapy", Symposium on

Biology of Gene Delivery, American Society of Gene Therapy 5th Annual Meeting,

Boston, MA. 06/02 "Intracellular Processing of rAAV and Implications for Gene Therapy", Symposium on

Biology of Gene Delivery, American Society of Gene Therapy 5th Annual Meeting,

Boston, MA. 06/02 "Cellular Targets for Gene Therapy of Cystic Fibrosis Lung Disease", Workshop at State

University of New York, Binghamton NY. 09/02 "Gene Therapy in Molecular Medicine: New Approaches and Clinical Hurdles",

International Conference on Wound Healing: Oxygen and Emerging Therapeutics, Columbus OH.

03/03 "Gene Therapy for Cystic Fibrosis: Moving Closer to the Target", University of

Washington, Seattle WA. 04/03 "Redox-Modulating Gene Therapies for Environmentally Induced Liver Injury", University

of Florida, Gainesville FL. 04/03 "Gene Therapy for Cystic Fibrosis: Moving Closer to the Target", University of Florida,

Gainesville FL. 04/03 "Redox-Modulating Gene Therapies for Environmentally Induced Liver Injury", University

of Arizona, Tucson AZ. 10/03 “Diversity of Redox Activation Pathway for NF-kappaB in Response to Environmental

Injuries: Applications in Liver-Directed Gene Therapies”, Iowa State University, Ames, IA 10/03 “The New Age of Gene Therapy in Molecular Medicine“, Iowa State University, Ames, IA


02/04 “Intracellular barriers to recombinant AAV transduction in polarized airway epithelia”, The Science of Viral Vectors for Gene Therapy, Gordon Research Center Conference, Santa Barbara, CA

04/04 “Gene Therapy for Cystic Fibrosis Lung Disease: Are we on Target?”, University of

Missouri-Columbia 04/04 “Stem Cells of Tracheal Epithelium”, Experimental Biology – Stem Cell Symposium,

Washington DC 06/04 “Intracellular Trafficking”, Educational Session Speaker, American Society of Gene

Therapy Meeting, Minneapolis, MN 07/04 “Lung Stem Cells”, FASEB Summer Research Conference, Saxtons River, Vermont 09/04 “Functional Expression of CFTR in Polarized Airway Epithelia with rAAV: Biologic

Barriers and Potential Solutions”, Cystic Fibrosis Foundation Therapeutics Gene Therapy for CF: Assessing the Possibilities, Bethesda MD

09/04 “Hybrid AAV Vectors with Non-Homologous ITRs Improves Directional Recombination

and Dual-Vector Reconstitution of Large Transgenes”, Xth Parvovirus Workshop, St. Petersburg, FL

10/04 “Recent Advances in Gene Therapy”. North American Cystic Fibrosis Conference, St.

Louis, MO 04/05 “Biology of Gene Therapy for Cystic Fibrosis Lung Disease”, University of Alabama at

Birmingham, Birmingham, AL. 04/05 “Progress Toward Gene Therapy for Cystic Fibrosis”, Johns Hopkins University,

Baltimore, MD 06/05 “Are Men Mice or Pigs? A Lung Gene Therapy and Cell Biology Perspective”, American

Society of Gene Therapy Meeting, St. Louis, MO. 10/05 “Splicesome-mediated RNA Trans-splicing with rAAV Partially Retores CFTR Function to

Polarized Human CF Airway Epithelial Cells”. North American Cystic Fibrosis Conference, Baltimore, MD.

11/05 “Species Choice for Animal Modeling Cystic Fibrosis and Gene Therapy to the Airway”.

Gene Therapy Symposia for Heart, Lung and Blood Diseases, Sonoma, CA. 12/05 “Progress Toward Gene Therapy for Cystic Fibrosis with Adeno-Associated Virus”,

Hospital for Sick Kids, Toronto, Canada 03/06 “Instracellular Trafficking of rAAV2”, Gordon Research Conference on The Science of

Viral Vectors for Gene Therapy, Ventura, California. 05/06 “Stem Cell Biology in the Adult Airways” (Scientific Symposium Speaker: “Respiratory

Tract: Lung Cell Biology Update – Identifying Gene Therapy Targets and Improving Gene Transfer Efficiency”), American Society of Gene Therapy Meeting, Baltimore, MD

05/06 “Writing Competitive Grants”, Educational Session Speaker, American Society of Gene

Therapy Meeting, Baltimore, MD 06/06 “Stem Cell Biology in the Adult Airways”, American Society of Gene Therapy Meeting,

Baltimore, MD


06/06 “Do we have the Vectors and Model Systems?’ Cystic Fibrosis Foundation Conference, Williamsburg, Virginia

06/06 “Stem Cell Niches in the Lung”, Ningixa University, China

10/06 “Nox2-Active Endosomes Facilitate Hydrogen Peroxide-dependent 1L-1 and TNF

Receptor Activation of NFB”, Gordon Research Conference, Les Diablerets, Switzerland 11/06 “Glandular Contributions to Innate Immunity in the Airway”, North American Cystic

Fibrosis Conference, Denver, Colorado 12/06 “Endosomal NADPH Oxidase Activation Facilitates Redox-dependent Responses to

Diverse Environmental Stimuli”, Superfund Annual Meeting, San Diego, California 12/06 “Nox-dependent Signaling by Redox Active Endosomes”, Redox Regulation of Signal

Transduction Meeting, Cold Spring Harbor, New York 01/07 “Subcellular Control of Redox-Dependent Inflammatory Signals by Early Endosomes”,

Case Western Reserve, Cleveland, Ohio 02/07 “Redox-dependent Endosomal Signaling by NADPH Oxidase”, University of Southern

California, Los Angeles, CA 02/07 “Redox-dependent Endosomal Signaling by NADPH Oxidase”, Department of

Neurosurgery Research Conference, The University of Iowa, Iowa City, IA 07/07 “Airway Development and Stem Cells”, NHLBI Resident Cellular Components of the

Human Lung Workshop, Bethesda, MD 09/07 "Airway Stem Cell Targets for Correction of the Basic Deficit", Thirteenth Broken Arrow

Conference and Lung Transplantation for Cystic Fibrosis Symposium, Quebec, Canada 08/07 "Ferreting out Effective Gene Therapies for Cystic Fibrosis", Children's Hospital of

Philadelphia, Philadelphia, PA 10/07 "Airway Submucosal Glands”, North American Cystic Fibrosis Conference, Anaheim, CA,

Roundtable 11/07 “Endosomal Regulation of NADPH Oxidases in Cell Signaling”, Society for Free Radical

Biology and Medicine, Washington DC 01/08 The Glandular Stem Cell Niche in Airway Development and Repair, Transatlantic Airway

Conference (TAC), Lucerne, Switzerland 03/08 “Endosomal Processing of rAAV2”, Gordon Research Conference, Ventura, CA 04/08 “Mechanisms of Endosomal Redox Signaling in Health and Disease: Lessons from

Amyotrophic Lateral Sclerosis”, University of Vermont, Burlington, VT. 04/08 “New Animal Models for Cystic Fibrosis Research”, European Cystic Fibrosis Society

Basic Science Conference, Douro, Portugal. 05/08 “Endosomal Redox Signaling in Health and Disease”, University of Pennsylvania Medical

Center, Philadelphia, PA. 06/08 “Endosomal Regulation of Nox2 in Health and Disease”, Gordon Research Conference,

New London, NH.


08/08 “The glandular stem/progenitor cell niche in airway development and repair”, Federation of American Societies for Experimental Biology, Saxtons River, Vermont.

08/08 “Molecular Regulation of NADPH Oxidases in Health and Disease”, Joint Seminar of the

38th Niigata Summer Seminar on Neuroscience and Integrative Brain Research

Symposium, Niigata University, Niigata, Japan. 09/08 “Molecular Regulation of NADPH Oxidases in Health and Disease”, Riken Brain Science

Institute, Riken, Japan. 11/08 “Molecular Regulation of NADPH Oxidases in Health and Disease”, Neurodegenerative

Disease Research Seminar, University of Michigan Medical School, Ann Arbor, MI. 12/08 “The Glandular Stem/Progenitor Cell Niche in Airway Development and Repair”,

University of Miami, Miami, FL. 02/09 “Regulation of NADPH Oxidase-Dependent Redoxosomal Signaling in Health and

Disease”, The Scripps Research Institute, LaJolla, CA. 06/09 “Phenotypic Analysis of a Ferret Model of Cystic Fibrosis”, European Coordination Action

for Research in Cystic Fibrosis, Erasmus MC Rotterdam. 10/09 “Phenotypic Analysis of a Ferret Model”, Jilin University, Changchun, Jilin, China 11/09 "Cytokine Signaling Through Redox Active Endosomes: The Redoxosome”, Wake

Forrest University, Winston-Salem, NC 04/10 “Phenotypic Analysis of a Ferret Model of Cystic Fibrosis Generated by Nuclear Transfer

Cloning” Cornell University, Ithaca, New York 04/10 “Characterization of a NEW CFTR Knockout Ferret Model” 2010 European Cystic

Fibrosis Society Conference, Carcavelos, Portugal 04/10 “Ferreting out cystic fibrosis disease biology in a new CFTR knockout model”,

Department of Physical Therapy Research Day, University of Iowa, Iowa City IA 05/10 “New Developments in Animal Models of Cystic Fibrosis”, Scientific Symposium Speaker,

13th Annual American Society of Gene and Cell Therapy Meeting, Washington DC

06/10 “Phenotypic Characterization of a CFTR Knockout Ferret Model”, 2

nd Biannual Cystic

Fibrosis Research Retreat, Philadelphia, PA 07/10 “Phenotypic Characterization of a CFTR Knockout Ferret Model”, Kansas State

University, Manhattan, Kansas 09/10 “Ferreting out cystic fibrosis disease biology in a new CFTR knockout model”. Cincinnati

Children's Hospital Medical Center, Cincinnati, OH 10/10 “Noah’s Ark of CF Animal Models: Updates and Discussion”, Brown Bag Session, 24

th

Annual North American Cystic Fibrosis Conference, Baltimore, MD 12/10 “Engineering Knockout and Transgenic Ferret Models”, Broad Institute, Cambridge, MA 02/11 “Genetic Modeling of Cystic Fibrosis in the Ferret”, Pediatric Grand Rounds, University of

Iowa Hospitals and Clinics, Iowa City IA


02/11 “Progress and Prospects: Techniques for Site-Directed Mutagenesis in Animal Models”, Gene Targeting International Conference, Vienna, Austria

02/11 “Submucosal gland responses to airway injury and disease”, Gordon Research

Conference, Ventura, CA 03/11 “Lung Disease and Diabetes in Ferrets with Cystic Fibrosis”, Johns Hopkins University,

Baltimore, MD 03/11 “Modeling Complexities of Disease and Gene Therapy in a Cystic Fibrosis Ferret Model

Generated by Somatic Cell Nuclear Transfer Cloning”, Los Angeles Minority Opportunities in Research Seminar Series, California State University

03/11 “University of Iowa: A Great Place for Graduate Education”, (Student Meeting), Los

Angeles Minority Opportunities in Research Seminar Series, California State University 04/11 “Lung Disease and Diabetes in CFTR-knockout Ferrets”, European Cystic Fibrosis Basic

Science Meeting, Tirrenia-Pisa, Italy 05/11 “Larger Animal Models of Cystic Fibrosis” (Session entitled “Scientific Symposium:

Clinical and Translational Studies for Genetic and Metabolic Diseases”), 14th Annual

American Society of Gene and Cell Therapy Meeting, Seattle, WA 05/11 “Ex Vivo Functional Analysis” (Session entitled “Functional Analysis of Stem Cells Getting

Ready to Translate”), at the American Thoracic Society International Conference, Denver, Co

06/11 “Pathogenesis of Early Inflammation in CF: Evidence from Large Animal Models”, 34

th

European Cystic Fibrosis Conference, Hamburg, Germany 07/11 “Cystic Fibrosis Related Diabetes and lung Disease in a Ferret Model”, Cardiovascular

Center Seminar, University of Iowa, Carver College of Medicine, Iowa City IA 11/11 “Alterations to Stem/Progenitor Cell Niches in the Cystic Fibrosis Airway”, Grand Rounds

Speaker, Respiratory Health and Regenerative Medicine Symposium, Hospital for Sick Children, Toronto, Ontario, Canada

11/11 “CF in Ferrets - Modeling CF Diabetes and Lung Disease” (Session entitled: Animal

Models: CF in Animals and Humans), North American CF Conference, Anaheim, California

04/12 “Early CFTR-Dependent Innate Immunity in the Ferret Lung” (Session entitled: Models of

CF Disease), European Cystic Fibrosis Society Basic Science Conference, Sainte-Maxime, France.

04/12 “Ferreting Out Mechanisms of Lung Disease and Gene Therapy for Cystic Fibrosis”,

Educational Research Seminar Series, Cystic Fibrosis Research and Translation Center and Research Development Program, University of Washington, Seattle Children’s Hospital

04/12 “Modeling Cystic Fibrosis Related Diabetes in the Ferret” Diabetes and Metabolism

Seminar Series, Diabetes Research Center, University of Washington 07/12 “CGRP in cystic fibrosis submucosal glands”, FASEB Science Research Conferences,

Saxtons River, Vermont


07/12 “Redoxosomes in Cytokine Mediated Signal Transduction” (Session entitled: Localized ROS and Redox Signaling), Gordon Research Conference Thiol-Based Redox Regulation and Signaling, Lewiston, Maine

10/12 “Animal Models of CFRD” (Session entitled: “Advances in CFRD Screening, Diagnosis

and Management”), North American CF Conference, Orlando, Florida 01/13 “Genetic Modeling of Lung Diseases in the Domestic Ferret”, presented at the University

of Washington, Seattle, WA 02/13 "Development and Maintenance of the Submucosal Gland Stem/Progenitor Cell Niche"

(Session entitled: “Specification, Differentiation and Patterning of Airway Epithelial Cell Lineages”) Keystone Stem Cell Meeting, Taos, NM

03/13 “CF Ferret Model” Annual ECFS Conference – New Frontiers in Basic Science of Cystic

Fibrosis, Malaga, Spain 09/13 “New Animal Models of Cystic Fibrosis” (Session entitled: Models and Tools for Studying

Chloride Transport in Health and Disease), 67th Annual Meeting and Symposium of the

Society of General Physiologists, Woods Hole, MA 09/13 “New Genetic Models for Cystic Fibrosis and Other Lung Disease” NHLBI Workshop

Animal Models of Lung Diseases, Washington DC 09/13 “Choosing a Species”, Session Chair, NHLBI Workshop Animal Models of Lung

Diseases, Washington DC 10/13 “CFRD: From Bench to Bedside and Back Again”, Cystic Fibrosis Advisory Board Track-

Plenary Session, North American CF Conference, Salt Lake City UT 12/13 “Boarding Noah's Ark with Cystic Fibrosis Animal Models”, presented at Boston

University, Boston, MA 01/14 “Ferreting Out Mechanisms of Disease in Cystic Fibrosis” presented at Vertex

Corporation, San Diego, CA 02/14 “Modeling Cystic Fibrosis Related Diabetes in the Ferret” presented at Institut de

recherches cliniques de Montreal (IRCM), Montreal, Canada 02/14 “Boarding Noah's Ark with Cystic Fibrosis Animal Models”, presented at the Distinguished

Seminar Speaker Series, National Jewish Health, Denver CO 04/14 “Discovery Science” presented at the International Society for Cellular Therapy, Paris,

France 05/14 “A novel chimeric adeno-associated virus 2/human bocavirus 1 parvovirus vector

efficiently transduces human airway epithelia” (Session entitled: AAV Vector Development) presented at the American Society of Gene and Cell Therapy, Washington DC

06/14 “Endocrine pancreas function at birth: lessons from the ferret” (Session entitled:

Multidisciplinary aspects of CFRD), presented at the European Cystic Fibrosis Conference, Gothenburg, Sweden

07/14 “Early Pathogenesis of Cystic Fibrosis Related Diabetes” presented at the Diabetes and

Obesity Seminar of the Fraternal Order of Eagles Diabetes Research Center, University of Iowa, Iowa City, Iowa


XVII. Other Comments

Patents Issued Adenovirus Vectors for Gene Therapy; Country of Filing: US; Patent #5,585,362; Issued date: 12/17/96 Model of Human Airway, Methods of Use; Country of Filing: US; Patent #5,667,766; Issued date: 9/16/97 Circular Intermediates of Adeno-Associated Virus for Gene Therapy; Country of Filing: US; Patent #6,436,392; Issue date: 8/20/02 Circular Intermediates of Adeno-Associated Virus for Gene Therapy; Country of Filing: US; Patent #6,897,045; Issue date: 5/24/05 Cellular Pathways for Enhancing rAAV Transduction (Tri-Peptides, Endosomal Processing); Country of Filing: Australia; Patent #782966; Issue date: 1/5/06 Cellular Pathways for Enhancing rAAV Transduction (Tri-Peptides, Endosomal Processing); Country of Filing: US; Patent #7,122,335; Issue date: 10/17/06 Use of AAV Intermolecular Recombination to Generate Cis-Acting AAV Vectors; Country of Filing: US; Patent #7,241,447; Issue date: 7/10/07 Circular Intermediates of Adeno-Associated Virus for Gene Therapy; Country of Filing: US; Patent #7,803,622; Issue date: 9/28/10 Transgenic Animal Models of Disease; Country of Filing: US; Patent #7,989,675; Issue date: 8/2/11 Cellular Pathways for Enhancing rAAV Transduction (Tri-Peptides, Endosomal Processing); Country of Filing: Japan; Patent #4,969,002; Issue date: 4/13/12 A New Class of Hybrid Adeno-Associated Virus Vectors With Non-homologous ITRs Improves Directional Recombination and Dual Vector Reconstitution of Large Transgenes; Country of Filing: US; Patent #8,241,622; Issue date: 8/14/12 Transgenic Porcine Models of Cystic Fibrosis; Country of filing: US; Patent #8,618,352 BS, Issue date: 12/31/13 Patents Filed (Still Active) Recombinant Human Bocavirus Type 1 Vector and Application; Country of Filing: US; Application date: 4/8/13

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University of Iowa · Research Skills (60:204), University of Iowa (2 lectures on grant writing), 2006-2008. Graduate Research Anatomy and Cell Biology (060:206), University of Iowa,