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Methylmalonyl-CoA Mutase

More information in Books or onNLM PubMed
Definition: An enzyme that catalyzes the conversion of methylmalonyl-CoA to succinyl-CoA by transfer of the carbonyl group. It requires a cobamide coenzyme. A block in this enzymatic conversion leads to the metabolic disease, methylmalonic aciduria. EC 5.4.99.2.  /defic: consider also METHYLMALONIC ACID /urine (IM) + AMINO ACID METABOLISM, INBORN ERRORS (IM)    Other names Methylmalonyl-CoA Isomerase; Mutase, Methylmalonyl-CoA; Methylmalonyl CoA Mutase; Methylmalonyl CoA Isomerase; Isomerase, Methylmalonyl-CoA
 
SubstanceCAS Registry & nameCategoriesSourceDrugs*
Methylmalonyl-Coenzyme A mutase deficiency  0   *Metabolism, Inborn Errors Methylmalonyl-CoA Mutase/deficiency.
3-carboxypropyl-coenzyme A  0   *Coenzyme A Methylmalonyl-CoA Mutase/antagonists & inhibitors. Eur J Biochem 1994 Nov 1;225(3):891-6

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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