CoA sequestrant
Coenzyme A sequestration
- Drug is activated to an acyl-CoA thioester, trapping free coenzyme A
- A starved mitochondrial CoA pool impairs β-oxidation and N-acetylglutamate synthesis
- Route to hyperammonemia
- No direct repletion exists — stopping the drug is the treatment
- Drug is activated to an acyl-CoA thioester, trapping free coenzyme A
- A starved mitochondrial CoA pool impairs β-oxidation and N-acetylglutamate synthesis
- Route to hyperammonemia; no direct repletion exists — stopping the drug is the treatment
Drugs with this mechanism (5)
- Valproic acid — Broad-spectrum anticonvulsant and mood stabilizer; highly teratogenic and metabolically burdensome.
- Divalproex DR — Delayed-release broad-spectrum antiseizure drug and mood stabilizer; highly teratogenic.
- Divalproex ER — Extended-release broad-spectrum antiseizure drug and mood stabilizer; highly teratogenic.
- Valproate sodium IV — Intravenous valproate for seizures when oral therapy is unavailable; broad-spectrum but highly teratogenic.
- Divalproex sprinkle — Valproate sprinkle capsule for seizures; useful when intact tablets cannot be swallowed.
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