Stereochemistry of the methylmalonyl-CoA decarboxylation reaction
نویسندگان
چکیده
منابع مشابه
The Absolute Configuration of Methylmalonyl Coenzyme A and Stereochemistry of the Methylmalonyl Coenzyme A Mutase Reaction*
Rropionyl coenzyme A was carboxylated with epimerasefree carboxylase, and the resulting methylmalonyl coenzyme A (a) was reduced with Raney nickel. Isolation of (-)(R)-P-hydroxyisobutyric acid N-phenylcarbamate (11) from the reaction mixture showed that methylmalonyl coenzyme A (a) has the (S) configuration. Propionyl coenzyme A was also converted to deuteriosuccinate with a DrO extract of a be...
متن کاملThe absolute configuration of methylmalonyl coenzyme A and stereochemistry of the methymalonyl coenzyme A mutase reaction.
Rropionyl coenzyme A was carboxylated with epimerasefree carboxylase, and the resulting methylmalonyl coenzyme A (a) was reduced with Raney nickel. Isolation of (-)(R)-P-hydroxyisobutyric acid N-phenylcarbamate (11) from the reaction mixture showed that methylmalonyl coenzyme A (a) has the (S) configuration. Propionyl coenzyme A was also converted to deuteriosuccinate with a DrO extract of a be...
متن کاملImportance of the histidine ligand to coenzyme B12 in the reaction catalyzed by methylmalonyl-CoA mutase.
Methylmalonyl-CoA mutase is an adenosylcobalamin (AdoCbl)-dependent enzyme that catalyzes the rearrangement of methylmalonyl-CoA to succinyl-CoA. The crystal structure of this protein revealed that binding of the cofactor is accompanied by a significant conformational change in which dimethylbenzimidazole, the lower axial ligand to the cobalt in solution, is replaced by His-610 donated by the a...
متن کاملNitric oxide inhibits mammalian methylmalonyl-CoA mutase.
Methylmalonyl-CoA mutase is a key enzyme in intermediary metabolism, and children deficient in enzyme activity have severe metabolic acidosis. We found that nitric oxide (NO) inhibits methylmalonyl-CoA mutase activity in rodent cell extracts. The inhibition of enzyme activity occurred within minutes and was not prevented by thiols, suggesting that enzyme inhibition was not occurring via NO reac...
متن کاملMethylmalonyl-CoA Epimerase Deficiency Mimicking Propionic Aciduria
Methylmalonyl-CoA epimerase (MCE) converts d-methylmalonyl-CoA epimer to l-methylmalonyl-CoA epimer in the propionyl-CoA to succinyl-CoA pathway. Only seven cases of MCE deficiency have been described. In two cases, MCE deficiency was combined with sepiapterin reductase deficiency. The reported clinical pictures of isolated MCE are variable, with two asymptomatic patients and two other patients...
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ژورنال
عنوان ژورنال: FEBS Letters
سال: 1987
ISSN: 0014-5793
DOI: 10.1016/0014-5793(87)80888-8