Specifications
Immunogen
A synthetic peptide of human PCCA is used for rabbit immunization.
Target
Alternative Names
PCCA; propionyl CoA carboxylase, alpha polypeptide; propionyl Coenzyme A carboxylase, alpha polypeptide; propionyl-CoA carboxylase alpha chain, mitochondrial; PCCase alpha subunit; pccA complementation group; propanoyl-CoA:carbon dioxide ligase alpha subu
Product Background
Antigen Description
The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Two transcript variants encoding different isoforms have been found for this gene.
Pathway
2-oxobutanoate degradation I, organism-specific biosystem; Fatty acid, triacylglycerol, and ketone body metabolism, organism-specific biosystem; Glyoxylate and dicarboxylate metabolism, organism-specific biosystem; Glyoxylate and dicarboxylate metabolism, conserved biosystem; Metabolic pathways, organism-specific biosystem; Metabolism, organism-specific biosystem; Metabolism of lipids and lipoproteins, organism-specific biosystem;
Citations
Publication ()
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