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, ...