The MCCC1 gene provides instructions for making one part (the alpha subunit) of an enzyme called 3-methylcrotonoyl-CoA carboxylase or MCC. These alpha subunits join with smaller beta subunits made from the MCCC2 gene; six of these pairings together form a functioning enzyme. The alpha subunit also includes a region for binding to the B vitamin biotin, which is required for the enzyme to function.
The MCC enzyme is found in mitochondria, which are the energy-producing centers inside cells. This enzyme plays a critical role in breaking down proteins obtained from food. Specifically, it is responsible for the fourth step in the breakdown of leucine, an amino acid that is a building block of many proteins. This step converts a molecule called 3-methylcrotonyl-CoA to a molecule called 3-methylglutaconyl-CoA. Additional chemical reactions convert 3-methylglutaconyl-CoA into molecules that are later used for energy.
Other Names
3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase alpha; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; MCCA; MCCase subunit alpha; MCCCalpha; MCCCα; methylcrotonoyl-CoA carboxylase 1; methylcrotonoyl-CoA carboxylase 1 alpha
Related Genetics Topics
Identifiers
- NCBI Gene: 56922
- OMIM: 609010
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
