Methylmalonyl-CoA mutase (MMUT)
Cofactors used
Clinical / pharmacological. Methylmalonic acidaemia; adenosylcobalamin (B12)-dependent
What it does, reaction by reaction
Propionyl-CoA to Succinyl-CoA (anaplerosis) Amino Acid & Nitrogen Metabolism · Mitochondrial matrix
L-Methylmalonyl-CoA -> succinyl-CoA
Converts L-Methylmalonyl-CoA into Succinyl-CoA
› Notes
Requires adenosylcobalamin. B12 deficiency raises serum methylmalonic acid, which distinguishes it from folate deficiency; inherited defects cause methylmalonic acidaemia.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
No regulation recorded for this enzyme in the source documents.
Recent literature
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