Trifunctional enzyme subunit alpha, mitochondrial
3-Hydroxyvalproic acid CoA → Pentanoyl-CoA
Isovaleryl-CoA dehydrogenase, mitochondrial
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Acyl-coenzyme A synthetase ACSM1, mitochondrial
NAD + Valproic acid → 4-ene-Valproic acid + NADH
Trifunctional enzyme subunit beta, mitochondrial
2-n-Propyl-4-oxopentanoic acid → 2-Propylsuccinic acid
4-Hydroxyvalproic acid → 2-n-Propyl-4-oxopentanoic acid
4-ene-Valproic acid → 2-Propyl-2,4-pentadienoic acid
Hydrogen Ion + NADPH + Oxygen + Valproic acid → 3-Hydroxyvalproic acid + NADP + Water
Hydrogen Ion + NADPH + Oxygen + Valproic acid → 5-Hydroxyvalproic acid + NADP + Water
Hydrogen Ion + NADPH + Oxygen + Valproic acid → 4-Hydroxyvalproic acid + NADP + Water
5-Hydroxyvalproic acid → 2-Propylglutaric acid
3-Hydroxyvalproic acid → 3-Oxovalproic acid
Valproic acid → 3-ene-valproic acid
2-ene-Valproic acid → 3-Hydroxyvalproic acid
Valproic acid → 2-ene-Valproic acid
NAD + Valproic acid CoA → 2-ene-Valproic acid CoA + NADH
2-ene-Valproic acid CoA + Water → 3-Hydroxyvalproic acid CoA
2-ene-Valproic acid CoA → 3-ene-Valproic acid CoA
3-ene-Valproic acid CoA → 2,3-diene-Valproic acid-CoA
3-Hydroxyvalproic acid CoA → Propionyl-CoA
Valproic acid CoA → Valproyl-dephospho-CoA
4-ene-Valproic acid CoA → 2,4-diene-Valproic acid-CoA
3-Hydroxyvalproic acid CoA + NADH → 3-oxo-Valproic acid CoA + NAD
4-ene-Valproic acid + Coenzyme A → 4-ene-Valproic acid CoA
Uridine diphosphate glucuronic acid + Valproic acid → Uridine 5'-diphosphate + Valproic acid glucuronide
Adenosine triphosphate + Coenzyme A + Valproic acid → Adenosine monophosphate + Pyrophosphate + Valproic acid CoA