Biotin cofactor: propionyl-CoA carboxylase (PCC)
- Feeds odd-chain fatty acids and some amino acids into the TCA cycle
- Propionyl-CoA → methylmalonyl-CoA; biotin carries the carboxyl
- Upstream partner of the B12-dependent methylmalonyl-CoA mutase step
Drugs with this mechanism (1)
- Biocytin — Biotin bound to lysine; biotinidase recycles it into free biotin during protein breakdown.
Self-check
- What does biotin-dependent propionyl-CoA carboxylation produce?
Methylmalonyl-CoA. - Which vitamin-dependent step follows this pathway?
The B12-dependent methylmalonyl-CoA mutase step.
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