MCCC1 wt Allele

**Semantic type:** Gene or Genome

**Definition:** Human MCCC1 wild-type allele is located in the vicinity of 3q27.1 and is approximately 101 kb in length. This allele, which encodes methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial protein, is involved in biotin binding and the metabolism of branched-chain amino acids. Mutation of the gene is associated with 3-methylcrotonylglycinuria caused by 3-methylcrotonyl-CoA carboxylase 1 deficiency.

**Synonyms:** - 3-Methylcrotonyl-CoA Carboxylase Biotin Containing Subunit Gene - 3-Methylcrotonyl-CoA Carboxylase, Biotin-Containing Subunit Gene - MCC-B - MCCA - MCCCalpha - Methylcrotonoyl-CoA Carboxylase 1 (Alpha) Gene - Methylcrotonoyl-CoA Carboxylase 1 Gene - Methylcrotonoyl-CoA Carboxylase Alpha Gene - Methylcrotonoyl-CoA Carboxylase, Alpha Gene - Methylcrotonoyl-Coenzyme A Carboxylase 1 (Alpha) Gene - Methylcrotonyl-CoA Carboxylase Subunit 1 wt Allele