Biotin cofactor: methylcrotonyl-CoA carboxylase (MCC)
- Required for leucine catabolism
- MCC deficiency is among the commonest findings on newborn screening
- Leucine catabolism's biotin step; MCC deficiency is a classic newborn-screen organic acidemia
Drugs with this mechanism (1)
- Biocytin — Biotin bound to lysine; biotinidase recycles it into free biotin during protein breakdown.
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