MCCC1 : methylcrotonyl-CoA carboxylase subunit 1
Description
The MCCC1 (methylcrotonyl-CoA carboxylase subunit 1) is a protein-coding gene located on chromosome 3.
The MCCC1 gene provides instructions for making the alpha subunit of the 3-methylcrotonyl-CoA carboxylase (MCC) enzyme. Six alpha subunits combine with six beta subunits (from the MCCC2 gene) to form a functional MCC enzyme. The alpha subunit contains a biotin-binding site, essential for enzyme function. MCC is located in mitochondria, where it breaks down dietary proteins. It specifically catalyzes the fourth step in leucine breakdown, converting 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA. This molecule is further processed to generate energy.
MCCC1 is also known as MCC-B, MCCA, MCCCalpha.
