Immunogen | Purifiedglialfilamentprotein(Debus,E.,1983). |
Host | Mouse |
Specificity | TheantibodyreactswithmouseGFAP.Intissuesectionsthis antibodystainsastrocytesandBergmangliacells(Debus,E.,1983). |
Isotype | IgG1 |
SpeciesReactivity | |
SpeciesReactivityNote | DemonstratedtoreactwithMouse.PredictedtoreactwithHuman,Rat,Porcine,andChickenbasedon100%sequencehomology. |
AntibodyType | MonoclonalAntibody |
EntrezGeneNumber | |
EntrezGeneSummary | Thisgeneencodesoneofthemajorintermediatefilamentproteinsofmatureastrocytes.Itisusedasamarkertodistinguishastrocytesfromotherglialcellsduringdevelopment.MutationsinthisgenecauseAlexanderdisease,araredisorderofastrocytesinthecentralnervoussystem.Alternativesplicingresultsinmultipletranscriptvariantsencodingdistinctisoforms.[providedbyRefSeq]. |
GeneSymbol | |
PurificationMethod | ProteinAPurfied |
UniProtNumber | |
UniProtSummary | FUNCTION:GFAP,aclass-IIIintermediatefilament,isacell-specificmarkerthat,duringthedevelopmentofthecentralnervoussystem,distinguishesastrocytesfromotherglialcells.
SIZE:432aminoacids;49880Da
SUBUNIT:Isoform3interactswithN-terminusofPSEN1.
SUBCELLULARLOCATION:Cytoplasm.Note=Associatedwithintermediatefilaments.
DISEASE:DefectsinGFAPareacauseofAlexanderdisease[MIM:203450].Alexanderdiseaseisararedisorderofthecentralnervoussystem.ItisaprogressiveleukoencephalopathywhosehallmarkisthewidespreadaccumulationofRosenthalfiberswhicharecytoplasmicinclusionsinastrocytes.Themostcommonformaffectsinfantsandyoungchildren,andischaracterizedbyprogressivefailureofcentralmyelination,usuallyleADIngtodeathusuallywithinthefirstdecade.InfantswithAlexanderdiseasedevelopaleukoencephalopathywithmacrocephaly,seizures,andpsychomotorretardation.Patientswithjuvenileoradultformstypicallyexperienceataxia,bulbarsignsandspasticity,andamoreslowlyprogressivecourse.
SIMILARITY:Belongstotheintermediatefilamentfamily. |
MolecularWeight | 50kDaObserved(UsingunconjugatedantibodyMAB3402) |