MCCC2 : methylcrotonyl-CoA carboxylase subunit 2


Description

The MCCC2 (methylcrotonyl-CoA carboxylase subunit 2) is a protein-coding gene located on chromosome 5.

The MCCC2 gene provides instructions for producing the beta subunit of the 3-methylcrotonyl-CoA carboxylase (MCC) enzyme, which is located in mitochondria. This enzyme is essential for breaking down proteins, particularly leucine, an amino acid. The MCC enzyme consists of six pairs of alpha and beta subunits, formed from the MCCC1 and MCCC2 genes respectively. Specifically, MCCC2 contributes to the fourth step in leucine breakdown, converting 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA. This product is further processed to generate energy.

The MCCC2 gene encodes the beta subunit of the 3-methylcrotonyl-CoA carboxylase enzyme, which catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA. This reaction is crucial for the breakdown of leucine and isovaleric acid.

MCCC2 is also known as MCCB, MCCCbeta.

Associated Diseases


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