<unk> -CoA<unk>  is a mitochondrial homodimer apoenzyme (EC. 5. 4.99.2) that focuses on the catalysis of<unk>  CoA to succinyl CoA. The enzyme is bound to adenosylcobalamin, a hormonal derivative of vitamin B12 in order to function.<unk>  is caused by genetic defect in the MUT gene responsible for encoding the enzyme. Deficiency in this enzyme accounts for 60% of the cases of methylmalonic acidemia.
Methylmalonyl-CoA mutase deficiency
Malonyl-CoA decarboxylase deficiency
Pyruvate kinase deficiency
3-Hydroxyisobutyryl-CoA deacylase deficiency
3-Methylcrotonyl-CoA carboxylase deficiency
Ornithine aminotransferase deficiency
2-Methylbutyryl-CoA dehydrogenase deficiency
Carbamoyl phosphate synthetase I deficiency