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This is my report on our cell biology. I hope it could help you. Objectives: Identify infectious proteins (PrPsc), difference of PrPc and PrPsc, list of neurodegenerative diseases that caused by prions.
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Neurodegenerative Diseases in Human and Animals
PRIONS
Muslima P. LiwalugMIB 1
ABSTRACT
PrPSc are infectious proteins that cause neurodegenerative diseases in humans and animals. Checking the signs of the disease is the best preliminary diagnosis and brain scan or blood test will determine the abnormal proteins that are present in the patient’s body. The result of the study determine the different kinds of neurodegenerative diseases that caused by PrPSc and how it affect the human and animals.
OBJECTIVESoTo determine Prions
oTo differentiate PrPC from PrPSc
oTo determine how it affects human and animal brains and the formation of holes
oTo determine the list of human and animal neurodegenerative diseases caused by PrPSc
PRIONS
o are not made up of cell
o proteins that are found
mostly abundant in the
brain
o doesn’t use energy
o doesn’t grow
o prions don’t die
o they reproduce via pre-
existing prions
ORIGIN OF PRIONS
All mammals appear to have prion protein genes and the gene sequences are similar, but not identical, in related species.
FUNCTION OF PRIONS
obinds with Cu (Copper)oAnti-oxidant activityoResistant to oxidative stressoPrevent neuronal dysfunction
oAbnormality of prion proteins can induce apoptosis of neurons.!
MATERIALS & METHODSChecking the List of
Signs of the Disease
Brain Scan via MRI or
EEG
Interpretation of the
Result
Blood Test
Interpretation of the
Result
SIGNS
Early Signs• Depression / anxiety• Insomnia• Dizziness• Altered mood• Unusual behavior• Tingling part of the
body
Later signs• Develops dementia• Loss of coordination• Visual disturbance
PS: Typical signs may not be present but problems with thinking skills or balance will certainly be noted.
BRAIN SCANS
Magnetic Resonance Imaging (MRI)
• reveals the characteristics patterns of brain degeneration that help diagnose PrPSc
Electroencephalogram (EEG)
• record the brain’s electrical pattern which can be particularly valuable because it shows specific type of abnormality in prion proteins
BLOOD TEST
Small amount of blood is mixed with special metal beads to which the PrPSc
sticks tightly.
Washed to removed PrPC and other blood components that could interfere with the test.
The amount of PrPSc attached to the beads is measured using newly developed antibodies that bind tightly to the prion proteins.
RESULTS
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Objectives:
TRANSFORMATIONS
PrPC
PrPSc
TRANSFORMATIONS
PrPC PrPSc
PROTEIN FOLDING BY CHAPERONS
Chaperon Proteins - provide a site where misfolded proteins can fold correctly
DIFFERENCE BETWEENPrPC and PrPSc
PrPC PrPSc
Solubility Soluble Non soluble
Structure Alpha-helix Beta-helix
Multimerisation Monomeric Multimeric
Infectivity Non infectious Infectious
Susceptibility to Proteinase K
Susceptible Resistant
INFECTIONo O – Origin of PRION
Contaminated meat
o Digestive Tracko Blood Streamo S – Spleeno N – Spleen to Brain o B – Brain
PATHOGENESIS
NEURODEGENERATIVE DISEASES CAUSED BY
PRIONSDisease Description
Infectious Diseases
Kuru • Human disease• Begins with a loss of coordination• followed by dementia
Scrapie • Disease of sheep and pigs• Characterized by intense itching in which animals
tend to scrape themselves into the trees• Followed by neurodegeneration
Mad cow disease (BSE) • Begins with changes in posture and temperament• Followed by loss of coordination and
neurodegeneration
Human Inherited Diseases
Creutzfeldt-Jakob disease • Characterized by loss of coordination and dementia
Gerstmann-Straussler-Scheinker disease
• Characterized by loss of coordination and dementia
Familial fatal insomnia • Begins w/ sleeping and ANS disturbances• Followed by insomnia and dementia
RESULT
BSESCRAPIE
RESULT
CONCLUSIONo Cook the meat thoroughly.
o If you are a raiser of cow, pig, sheep or a goat, check the list of the signs of prion diseases.
o If you think you have the early signs of the disease, immediately consult to a doctor.
References:1. McKintosh, E. et.al, 2003. Prion Diseases
2. Collinge, J., 2001. Prion Diseases of Humans and Animals: Their Causes and Molecular Basis
3. Belay, E.D., 1999. Transmissible Spongiform Encephalopathies in Humans
4. Belay, E.D. and Schonberger, L.B., 2005. The Public Health Impact of Prion Diseases
5. George-Hyslop P., McLaurin, J., and Westaway, D., 2006. Human Prion Diseases and Protein Misfolding Disorders
6. Veith, N.M., 2008. Cellular Trafficking of the Pathogenic Prion Protein PrPSc and Phenotypic Characterization of Deletion Mutants in the Hydrophobic Domain of the Normal Prion ProteinC
7. Marsh, D., 2002. Mad Cow Disease: The Risks to Humans
8. Weissmann, C. et.al, 2002. Molecular Biology of Prions
9. Mahmoud, M.K.H., 2009. Studies on Pathogenic Mechanisms of Prion Diseases and Evaluation of Prion Strains Properties
10. Aguzzi, A., et.al, 2007. Molecular Mechanisms of Prion Pathogenesis
Thank You!
HAPPY VALENTiNE’S
DAY
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