Description | |
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CatalogueNumber | MAB1964 |
BrandFamily | Chemicon® |
TradeName |
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Description | Anti-Integrinβ4Antibody,clone3E1 |
AlternateNames |
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BackgroundInformation | Integrinbeta4isaglycoproteinwhichassociateswiththea6integrintoformthea6/b4complex.Integrinalpha6/beta4isareceptorforlaminin.Itplaysacriticalstructuralroleinthehemidesmosomeofepithelialcells.DefectsinIntegrinbeta4geneareacauseofepidermolysisbullosaletaliswithpyloricatresia(EBPA);alsoknownasjunctionalepidermolysisbullosawithpyloricatresia(PA-JEB)oraplasiacutiscongenitawithgastrointestinalatresia.EB-PAischaracterizedbymucocutaneousfragilityandgastrointestinalatresia,whichmostcommonlyaffectsthepylorus.Moreover,defectsinIntegrinbeta4geneareacauseofgeneralizedatrophicbenignepidermolysisbullosa(GABEB).Thisnonlethalformofjunctionalepidermolysisbullosaischaracterizedbylifelongblisteringoftheskin,associatedwithhairandtoothabnormalities. |
ProductInformation | |
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Format | Ascites |
Control |
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Presentation | UnPurifiedascitescontainingnopreservatives. |
StorageandShippingInformation | |
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StorageConditions | Maintainfor2yearsat-20°Cfromdateofshipment.Aliquottoavoidrepeatedfreezingandthawing.Formaximumrecoveryofproduct,centrifugetheoriginalvialafterthawingandpriortoremovingthecap. |
BIOLOGicalInformation | |
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Clone | 3E1 |
Concentration | PleaserefertotheCertificateofAnalysisforthelot-specificconcentration. |
Host | Mouse |
Specificity | ReactswithHumanbeta4integrin.Theantigenispresentonepithelialcells. |
Isotype | IgG1 |
SpeciesReactivity |
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AntibodyType | MonoclonalAntibody |
EntrezGeneNumber | |
EntrezGeneSummary | Integrinsareheterodimerscomprisedofalphaandbetasubunits,thatarenoncovalentlyassociatedtransmembraneglycoproteinreceptors.Differentcombinationsofalphaandbetapolypeptidesformcomplexesthatvaryintheirligand-bindingspecificities.Integrinsmediatecell-matrixorcell-celladhesion,andtransducedsignalsthatregulategeneexpressionandcellgrowth.Thisgeneencodestheintegrinbeta4subunit,areceptorforthelaminins.Thissubunittendstoassociatewithalpha6subunitandislikelytoplayapivotalroleinthebiologyofinvasivecarcinoma.Mutationsinthisgeneareassociatedwithepidermolysisbullosawithpyloricatresia.Multiplealternativelysplicedtranscriptvariantsencodingdistinctisoformshavebeenfoundforthisgene. |
GeneSymbol |
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PurificationMethod | Unpurified |
UniProtNumber | |
UniProtSummary | FUNCTION:SwissProt:P16144#Integrinalpha-6/beta-4isareceptorforlaminin.Itplaysacriticalstructuralroleinthehemidesmosomeofepithelialcells. SIZE:1822aminoacids;202151Da SUBUNIT:Heterodimerofanalphaandabetasubunit.Beta-4associateswithalpha-6. SUBCELLULARLOCATION:Membrane;Single-passtypeImembraneprotein. TISSUESPECIFICITY:Integrinalpha-6/beta-4ispredominantlyexpressedbyepithelia.Isoformbeta-4Disalsoexpressedincolonandplacenta.Isoformbeta-4Eisalsoexpressedinepidermis,lung,duodenum,heart,spleenandstomach. DOMAIN:SwissProt:P16144Thefibronectintype-III-likedomainsbindBPAG1andplectinandprobablyalsorecruitBP230. DISEASE:SwissProt:P16144#DefectsinITGB4areacauseofepidermolysisbullosaletaliswithpyloricatresia(EB-PA)[MIM:226730];alsoknownasjunctionalepidermolysisbullosawithpyloricatresia(PA-JEB)oraplasiacutiscongenitawithgastrointestinalatresia.EB-PAischaracterizedbymucocutaneousfragilityandgastrointestinalatresia,whichmostcommonlyaffectsthepylorus.&DefectsinITGB4areacauseofgeneralizedatrophicbenignepidermolysisbullosa(GABEB)[MIM:226650].Thisnonlethalformofjunctionalepidermolysisbullosaischaracterizedbylife-longblisteringoftheskin,associatedwithhairandtoothabnormalities. SIMILARITY:Belongstotheintegrinbetachainfamily.&Contains1Calx-betadomain.&Contains4fibronectintype-IIIdomains.&Contains1VWFAdomain. |
PhysicochemicalInformation |
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Dimensions |
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MaterialsInformation |
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MaterialsInformation |
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