MCCC1: Proteins and Enzymes

MCCC1, also known as Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, is a 725 amino acid protein that is 80 kDa, located in mitochondrion matrix, functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Studies on this protein have shown a relationship with the following diseases and disorders: 3 methylcrotonyl-coa carboxylase 1 deficiency, pseudomyxoma peritonei, mucinous cystadenocarcinoma, cystadenocarcinoma, 3-methylcrotonyl-coa carboxylase deficiency, carnitine deficiency, neisseria meningitides, Parkinson's disease, hypotonia, pneumonia, and tuberculosis. This protein has also been shown to have interactions with PPP2R2B, YWHAB, ACADM, AUH and ECHS1 in valine, leucine and isoleucine degradation, metabolic pathways, metabolism of amino acids and derivatives, and branched-chain amino acid catabolism pathways.