pinewood scientific flaer paroxsysmal nocturnal hemoglobinuria
TRANSCRIPT
Pinewood Scientific
Paroxsysmal 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
Distributed by:
Cedarlane Laboratories
www.cedarlanelabs.com
In Canada: Toll Free: 1-800-268-5058
In the U.S.A.: Toll Free: 1-800-721-1644
Distributed by:
Cedarlane Laboratories
www.cedarlanelabs.com
In Canada: Toll Free: 1-800-268-5058
In the U.S.A.: Toll Free: 1-800-721-1644www.cedarlanelabs.com
ISO 9001 and ISO 13485 registered.
www.cedarlanelabs.comISO 9001 and ISO 13485 registered.
FLAER
Distributed By:Please contact youAr local CEDARLANE disAtributor or
visit our website foAr more information.
www.cedarlanelabs.comISO 9001 and ISO 1A3485 registered.
FLAER (Alexa 488 proaerolysin variant)
Format Size Est. # of Tests Cat #
Powder 25ug 50 CL-FL1
Powder 50ug 100 CL-FL2
Liquid 25ug 50 CL-FL1S
Liquid 50ug 100 CL-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) Brminging wider meaningm 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.
Ordering FLAER:
FLAERisdistributedinNorthAmericabyCedarlaneLaboratoriesLimited
Pleaseseewebsiteforalistofinternationaldistributorswww.cedarlanelabs.com/flcaer
SolidFLAERcanalsobeordereddirectlyfromPinewoodScientificServicesInc.
09/09PricesarelistedinUSdollarsandaresubjecttochangewithoutnotice
Liquid FLAER and reconstituted improvedpowdered FLAER are equivalent. Both retain strong signals after aAt least 4 months when storAed at 4°C.
www.dlongwood.comCamino del Pilón,86 Casa- 750011- Zaragoza e-mail: [email protected] Tel. 976 320 638 Fax.976 320 660