PCCB wt Allele

**Semantic type:** Gene or Genome

**Definition:** Human PCCB wild-type allele is located in the vicinity of 3q22.3 and is approximately 88 kb in length. This allele, which encodes propionyl-CoA carboxylase beta chain, mitochondrial protein, is involved in the carboxylation of butynyl-CoA or propionyl-CoA and the catabolism of various fatty acids and branched-chain amino acids. Mutations in the gene are associated with propionic acidemia type 2.

**Synonyms:** - PccB Complementation Group Gene - Propionyl CoA Carboxylase, Beta Polypeptide Gene - Propionyl Coenzyme A Carboxylase, Beta Polypeptide Gene - Propionyl-CoA Carboxylase Beta Subunit Gene - Propionyl-CoA Carboxylase Subunit Beta wt Allele - Propionyl-CoA Carboxylase, Beta Subunit Gene