Methylmalonyl-CoA-Mutase

  • OMIM: 609058
  • UniProt: P22033
  • MGI: 97239

Methylmalonyl CoA mutase (MCM ) is an enzyme that is required in the metabolism of any living being to reduce a plurality of amino acids, fatty acids and cholesterol. MCM catalyzes the reaction step of L- methylmalonyl -CoA to succinyl -CoA, a rearrangement; Therefore, MCM is one of the isomerases. In some bacteria, it is part of the structure of propionate from tricarboxylic acid cycle intermediates. In eukaryotes MCM is localized in the mitochondria. Lack of MCM in humans leads to hereditary and often fatal methylmalonic acidemia.

Catalyzed reaction

  

L- methylmalonyl -CoA is rearranged to succinyl -CoA. In bacteria, the reaction proceeds in the opposite direction.

Human Genome Organisation Mouse Genome Informatics Methylmalonyl-CoA Succinyl-CoA
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