The MCCC2 gene provides instructions for making one part (the beta subunit) of an enzyme called 3-methylcrotonoyl-CoA carboxylase or MCC. These beta subunits join with larger alpha subunits made from the MCCC1 gene; six of these pairings together form a functioning enzyme.
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 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA carboxylase, beta; MCCase subunit beta; MCCB; MCCCbeta; MCCCβ; methylcrotonoyl-CoA carboxylase 2; methylcrotonoyl-CoA carboxylase beta; methylcrotonoyl-Coenzyme A carboxylase 2 (beta)
Related Genetics Topics
Identifiers
- NCBI Gene: 64087
- OMIM: 609014
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
