The protein encoded by this gene is the alpha subunit ofnthe heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase.nPCCA encodes the biotin-binding region of this enzyme. Mutations inneither PCCA or PCCB (encoding the beta subunit) lead to an enzymendeficiency resulting in propionic acidemia. Multiple transcriptnvariants encoding different isoforms have been found for this gene.
应用类型
WB,IHC
免疫原
This PCCA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 362-390 amino acids from the Central region of human PCCA.
