The enzyme (S)-methylmalonyl-CoA hydrolase (EC 3.1.2.17) catalyzes the reaction
The enzyme characterised from liver hydrolyses (S)-methylmalonyl-CoA to coenzyme A and methylmalonic acid, which forms the thioester in the starting material. The starting material is produced from propionyl-CoA, a breakdown product of some amino acids, and an increase of methylmalonic acid is a marker of vitamin B12 deficiency as methylmalonyl-CoA is converted to succinyl-CoA by an enzyme using the vitamin as a cofactor.
This enzyme is a hydrolase, specifically one acting on thioester bonds. The systematic name of this enzyme class is (S)-methylmalonyl-CoA hydrolase. This enzyme is also called D-methylmalonyl-coenzyme A hydrolase.
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