… Among them, methylmalonyl-CoA mutase (MCM) has been extensively studied. This
enzyme catalyzes the reversible isomerization of L-methylmalonyl-CoA to succinyl-CoA using …

Methylmalonic acidemia/aciduria (MMA) is a genetically heterogeneous group of inherited
metabolic disorders biochemically characterized by the accumulation of methylmalonic acid. …

… We have proposed that the accumulation of methylmalonylCoA, which … of this hypothesis,
we have examined the effect of methylmalonyl-CoA … It was shown that methylmalonyl-CoA …

… Synthetic propionyl CoA2 and methylmalonyl CoA3 were prepared as previously described.
Since synthetic methylmalonyl CoA is a racemic mixture of two stereoisomers, only one of …

… that methylmalonyl coenzyme A (a) has the (S) configuration. … It was concluded that (S)-methylmalonyl
coenzyme A, formed … methylmalonyl(coenzyme A)-2-D, and that (R)-methylmalonyl…

Publisher Summary This chapter discusses the preparation and purification of methylmalonyl
coenzyme A. Two methods have been reported for the preparation of the monothioester of …

… We conclude that the clinical spectrum of methylmalonyl-CoA mutase deficiency is wider …
deficient activity of methylmalonyl-CoA mutase, an enzyme that converts methylmalonyl-CoA to …

Methylmalonyl coenzyme A (CoA) mutase has been purified to apparent homogeneity from
human liver by a procedure involving column chromatography on DEAE-cellulose, Matrex-…

… Intramolecularly doubly labeled methylmalonyl-CoA was … methylmalonyl-CoA and unlabeled
methylmalonyl-CoA were combined and then converted to succinyl-CoA by methylmalonyl …

X-ray crystal structures of methylmalonyl-CoA mutase in complexes with substrate
methylmalonyl-… explains the stereochemical selectivity of the enzyme for (2R)-methylmalonyl-CoA. …