Collagenalpha-3(IV)chain,Goodpastureantigen,COL4A3,GlomerularBasalMembrane,GBM.
TypeIVcollagenisamajorstructuralcomponentofbasementmembranes.Itisamultimericproteincomposedof3alphasubunits,whichareencodedby6differentgenes,alpha1throughalpha6.Eachofthesealphasubunitscanformatriplehelixstructurewith2othersubunitstoformtypeIVcollagen.TheGoodpasturesyndromeisaconditioninwhichautoantibodiesbindtothecollagenmoleculesinthebasementmembranesofalveoliandglomeruli.Theepitopesthatelicittheseautoantibodiesarerestrictedbasicallytothenon-collagenousC-terminaldomainoftheprotein.TherearenumerousalternativetranscriptsthatappeartobeuniquetothehumanCOL4A3andalternativesplicingislimitedtothesixexonsthatencodethisC-terminaldomain.COL4A3isalsolinkedtoanautosomalrecessiveformofAlportsyndrome.ThemutationscontributingtotheAlportsyndromearealsosituatedwithintheexonsthatencodethisC-terminalregion.COL4A3isorganizedinahead-to-headconformationwithanothertypeIVcollagengenesothateachgenepairsharesacommonpromoter.SeveralexonsofCOL4A3areinterspersedwithexonsofanuncharacterizedgenewhichisontheoppositestrand.
Humanα3chainofcollagenIV;identicalwiththeantigencalled"glomerularbasalmembraneantigen"(GBM).CalculatedMolecularweight:48kDa.Calculatedisoelectricpoint:pH8.9.CDNAcodingforaminicollagenversionofthehumancollagenIVα3chainfusedtoa6-histidinepurificationtag.Thetermminicollagendesignatestheremovalofmostoftheepitope-lesstriplehelicalcollagenousregion(situatedbetweentheN-terminal7SdomainandtheC-terminalnoncollagenousNC1domain),whichisarequirementforrecombinantproductionofthisantigen.
Sf9insectcells.
SterileFilteredclearsolution.
COL4A3Humanbiotincontains20mMHEPES,pH7.5,0.02%SDSand0.01mMEDTA.
Storeat4°Cifentirevialwillbeusedwithin2-4weeks.
Store,frozenat-20°Cforlongerperiodsoftime.
Avoidmultiplefreeze-thawcycles.
Greaterthan95%asdeterminedbyCapillaryElectrophoresis.
1.BindsIgGtypehumanautoantibodies.2.FunctionalStreptavidinbasedELISAtest(analysisofpositive/negativesamples.)
IthasbeenreportedthattheimmunodominantepitopeofCOL4A3isacrypticepitopethatisnoteasilyaccess
IBLetothecorrespondingautoantibodies.Itisnecessarytotreattheproteinundernonreducingconditionswithadenaturantsuchasureatounmasktheepitopes(seeHellmarketal.inAutoantibodies,Peter,J.B.andShoenfeld,Y.,eds.,ElsevierB.V.,1996,pp291-298).
Prospec"sproductsarefurnishedforLABORATORYRESEARCHUSEONLY.Theproductmaynotbeusedasdrugs,agriculturalorpesticidalproducts,foodadditivesorhouseholdchemicals.