Malonyl-CoA semialdehyde ↔ 3-Hydroxypropionyl-CoA
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
Acetyl-CoA + Adenosine triphosphate + Hydrogen carbonate → Adenosine diphosphate + Malonyl-CoA + Phosphoric acid
Malonyl-CoA ↔ Malonyl-CoA semialdehyde
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Dihydrolipoyl dehydrogenase, mitochondrial
L-Valine → (S)-Methylmalonic acid semialdehyde
Propionyl-CoA carboxylase alpha chain, mitochondrial
Malonyl-CoA decarboxylase, mitochondrial
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
Acetyl-CoA acetyltransferase, mitochondrial
4-aminobutyrate aminotransferase, mitochondrial
Acyl-CoA synthetase short-chain family member 3
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD + Water → Hydrogen carbonate + NADH + Propionyl-CoA
S-Methylmalonyl-CoA → R-Methylmalonyl-CoA
Valine, Leucine, and Isoleucine Degradation
2 Acetyl-CoA ↔ Acetoacetyl-CoA + Coenzyme A
Propionyl-CoA carboxylase beta chain, mitochondrial
Glycine, Serine, and Threonine Metabolism
Oxoglutaric acid + β-Alanine → L-Glutamic acid + Malonic semialdehyde
Malonyl-CoA → Acetyl-CoA + Carbon dioxide
3-Hydroxypropionyl-CoA ↔ Acrylyl-CoA + Water
3-Hydroxypropionyl-CoA + Water ↔ Coenzyme A + Hydroxypropionic acid
Acrylyl-CoA + electron-transfer flavoprotein ↔ Propionyl-CoA + Reduced electron-transfer flavoprotein
Adenosine monophosphate + Propionyl-CoA + Pyrophosphate ↔ Adenosine triphosphate + Pantetheine + Propinol adenylate
Adenosine triphosphate + Pantetheine + Propinol adenylate ↔ Adenosine monophosphate + Propionyl-CoA + Pyrophosphate
Propinol adenylate ↔ Adenosine monophosphate + Propionic acid
Coenzyme A + Malonic semialdehyde + NAD → Acetyl-CoA + Carbon dioxide + NADH
Methylmalonic Aciduria Due to Cobalamin-Related Disorders
Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Hydrogen + S-Methylmalonyl-CoA
2-Ketobutyric acid + Coenzyme A + NAD → NADH + Propionyl-CoA
2-Hydroxybutyric acid + NAD ↔ 2-Ketobutyric acid + Hydrogen Ion + NADH
branched-chain alpha-keto dehydrogenase complex
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH