Biotin cofactor: methylcrotonyl-CoA carboxylase (MCC)
Biotin cofactor: methylcrotonyl-CoA carboxylase (MCC) — mechanism card
- Required for leucine catabolism
- MCC deficiency is among the commonest findings on newborn screening
Drugs with this mechanism (1)
- Biocytin — Biotin bound to lysine; biotinidase recycles it into free biotin during protein breakdown.
Self-check
- Which amino acid requires MCC for catabolism?
Leucine. - Where is MCC deficiency commonly detected?
Newborn screening.
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