Methylmalonyl-CoA mutase cofactor
- The mitochondrial arm of vitamin B12
- Converts methylmalonyl-CoA to succinyl-CoA, feeding odd-chain fats into the TCA (tricarboxylic acid) cycle
- Required cofactor for methylmalonyl-CoA mutase
- Deficiency → methylmalonic acid accumulation — the lab clue that distinguishes B12 from folate deficiency
Drugs with this mechanism (1)
- Adenosylcobalamin — Active vitamin B12; methylmalonyl-CoA mutase cofactor.
Self-check
- What does the mitochondrial B12 pathway convert methylmalonyl-CoA into?
Succinyl-CoA. - Which accumulating metabolite distinguishes B12 from folate deficiency?
Methylmalonic acid.
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