Pinewood Scientific

P a r o x y s m a l Nocturnal Hemoglobinuria

• Alexa fluor® 488 labelled reagent

• More sensitive than CD59;

detects small, abnormal granulocyte

populations to a level of 0.5%

• Now available in stabilized

liquid and lyophilized formats

FLAER

Distributed By:

www.cedarlanelabs.comISO 9001 and ISO 13485 registered.

FLAER (Alexa 488 proaerolysin variant)

Format Size Est. # of Tests Cat #

Powder 25ug 50 FL1

Powder 50ug 100 FL2

Liquid 25ug 50 FL1S

Liquid 50ug 100 FL2S

TheproductsareforResearchUseOnly

PNH: A progressive, destructive, and life-threatening disease

Paroxysmalnocturnalhemoglobinuria(PNH)isastemcell

disordercausedbyamutationofageneinvolvedinthesyn-

thesisoftheGPI(glycosylphosphatidylinositol)anchorofa

groupofsurfaceproteinsoncirculatingcells.Affectedcells

aresensitivetocomplement-mediatedhemolysisandthis

mayleadtolife-threateningthrombosis,chronickidneydis-

ease,pulmonaryhypertension,endorgandamage,ischemic

boweldisease,hepatic failure,andanemia—allofwhich

contributetoashortenedlifespanforpatientswithPNH.

IdentifyingPNHpatientsearlyinthecourseoftheirdisease

mayofferthebestopportunityforlong-termmanagement.

Inthepast,PNHhasbeenchallengingtoidentifyeffective-

ly.However,inrecentyearsimpressivestrideshavebeen

madeinunderstandingofPNHpathology,accompaniedby

greatlyimproveddetectiontechniques,includingmultipara-

metricflowcytometryusingFLAER.

About FLAER

FLAER is an Alexa®488 labeled variant of aerolysin, a

uniqueproteinthatbindstightlyandspecificallytomam-

malian GPI anchors. FLAER will not bind to PNH cells

becausetheydonotproducetheanchor.BeforeFLAER,

detectionofPNHclonesbyflowcytometryreliedonfluo-

rescentlylabeledantibodiestoGPI-linkedproteinssuchas

CD59andCD55.Theseantibodiesdonotbindwithhigh

affinity,sothatsmallPNHclonesarenotdetected.Also,

they each screen for the absence of a specific protein,

ratherthanlossoftheGPIanchor,andthereforethereis

theriskoffalsenegativeresults.SinceFLAERbindstothe

GPIanchor itself,onlyPNHcells,whichlacktheanchor,

willbenegative.AndsinceFLAERbindswithhighaffinity,

verysmallPNHpopulationscanbedetected.

ReferencesArecentbriefreviewofthestatusandimportanceofPNHandthekeyroleofFLAERindetectionbyflowcytometry:

Titus, K. (2009) Bringing wider meaning to a rare disease. Cap Today. January

ArecentpublishedmethodforPNHdetectionusingFLAER:

Sutherland et al. (2007) Diagnosing PNH with FLAER and multiparameter flow cytometry. Cytometry Part B (Clinical Cytometry) 72B: 167-177

Adetailedprotocolisavailableuponrequest.

AlexaFluor®isaregisteredtrademarkofMolecularProbes,Inc.

Liquid FLAER and reconstituted improved powdered FLAER are equivalent. Both retain strong signals after at least 4 months when stored at 4°C.