ENCODES a protein that exhibits methylcrotonoyl-CoA carboxylase activity; INVOLVED IN coenzyme A metabolic process; PARTICIPATES IN 3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway; 3-hydroxyisobutyric aciduria pathway; 3-methylcrotonyl CoA carboxylase 1 deficiency pathway; ASSOCIATED WITH 3-Methylcrotonyl-CoA carboxylase 2 deficiency (ortholog); 3-Methylcrotonyl-CoA carboxylase deficiency (ortholog); mitochondrial metabolism disease (ortholog); FOUND IN methylcrotonoyl-CoA carboxylase complex (ortholog); mitochondrial matrix (ortholog); mitochondrion (ortholog); INTERACTS WITH (+)-schisandrin B; 2,4-dinitrotoluene; 2,6-dinitrotoluene