SpecificationsSPECIESREACTIVITY:Human,Mouse,RatTESTEDAPPLICATIONS:ELISA,WBAPPLICATIONS:ABCD4antibodycanbeusedfordetectionofABCD4byELISAat1:312500.ABCD4antibodycanbeusedfordetectionofABCD4bywesternblotat1μg/mL,andHRPconjugatedsecondaryantibodyshouldbediluted1:50,000-100,000.USERNOTE:Optimaldilutionsforeachapplicationtobedeterminedbytheresearcher.POSITIVECONTROL:1)721_BCellLysatePREDICTEDMOLECULARWEIGHT:68kDaIMMUNOGEN:AntibodyproducedinrabbitsimmunizedwithasyntheticpeptidecorrespondingaregionofhumanABCD4.HOSTSPECIES:RabbitPropertiesPURIFICATION:Antibodyispurifiedbypeptideaffinitychromatographymethod.PHYSICALSTATE:LyophilizedBUFFER:AntibodyislyophilizedinPBSbufferwith2%sucrose.Add50μLofdistilledwater.Finalantibodyconcentrationis1mg/mL.CONCENTRATION:1mg/mlSTORAGECONDITIONS:Forshortperiodsofstorage(days)storeat4˚C.Forlongerperiodsofstorage,storeABCD4antibodyat-20˚C.Aswithanyantibodyavoidrepeatfreeze-thawcycles.CLONALITY:PolyclonalCONJUGATE:UnconjugatedAdditionalInfoALTERNATENAMES:ABCD4,ABC41,EST352188,P70R,P79R,PMP69,PXMP1L,MAHCJACCESSIONNO.:NP_005041PROTEINGINO.:4826958OFFICIALSYMBOL:ABCD4GENEID:5826BackgroundBACKGROUND:ABCD4isamemberofthesuperfamilyofATP-bindingcassette(ABC)transporters.ABCproteinstransportvariousmoleculesacrossextra-andintra-cellularmembranes.ABCgenesaredividedintosevendistinctsubfamilies(ABC1,MDR/TAP,MRP,ALD,OABP,GCN20,White).ThisproteinisamemberoftheALDsubfamily,whichisinvolvedinperoxisomalimportoffattyacidsand/orfattyacyl-CoAsintheorganelle.AllknownperoxisomalABCtransportersarehalftransporterswhichrequireapartnerhalftransportermoleculetoformafunctionalhomodimericorheterodimerictransporter.Thefunctionofthisperoxisomalmembraneproteinisunknown.However,itisspeculatedthatitmayfunctionasaheterodimerforanotherperoxisomalABCtransporterand,therefore,maymodifytheadrenoleukodystrophyphenotype.Itmayalsoplayaroleintheprocessofperoxisomebiogenesis.TheproteinencodedbythisgeneisamemberofthesuperfamilyofATP-bindingcassette(ABC)transporters.ABCproteinstransportvariousmoleculesacrossextra-andintra-cellularmembranes.ABCgenesaredividedintosevendistinctsubfamilies(ABC1,MDR/TAP,MRP,ALD,OABP,GCN20,White).ThisproteinisamemberoftheALDsubfamily,whichisinvolvedinperoxisomalimportoffattyacidsand/orfattyacyl-CoAsintheorganelle.AllknownperoxisomalABCtransportersarehalftransporterswhichrequireapartnerhalftransportermoleculetoformafunctionalhomodimericorheterodimerictransporter.Thefunctionofthisperoxisomalmembraneproteinisunknown.However,itisspeculatedthatitmayfunctionasaheterodimerforanotherperoxisomalABCtransporterand,therefore,maymodifytheadrenoleukodystrophyphenotype.Itmayalsoplayaroleintheprocessofperoxisomebiogenesis.Alternativesplicingresultsinatleasttwodifferenttranscriptvariants,onewhichisprotein-codingandonewhichisprobablynotprotein-coding.REFERENCES:1)Asheuer,M.,(2005)Hum.Mol.Genet.14(10),1293-1303.