Malonyl-CoA semialdehyde ↔ 3-Hydroxypropionyl-CoA
Malonyl-CoA ↔ Malonyl-CoA semialdehyde
L-Valine → (S)-Methylmalonic acid semialdehyde
4-aminobutyrate aminotransferase, mitochondrial
2 Acetyl-CoA ↔ Acetoacetyl-CoA + Coenzyme A
branched-chain alpha-keto dehydrogenase complex
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
Oxoglutaric acid + β-Alanine → L-Glutamic acid + Malonic semialdehyde
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
Propionyl-CoA carboxylase beta chain, mitochondrial
Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
Glycine, Serine, and Threonine Metabolism
Ubiquitin-conjugating enzyme E2 variant 3
Alpha-aminoadipic semialdehyde dehydrogenase
Valine, Leucine, and Isoleucine Degradation
Coenzyme A + Malonic semialdehyde + NAD → Acetyl-CoA + Carbon dioxide + NADH
Methylmalonic Aciduria Due to Cobalamin-Related Disorders
2-Hydroxybutyric acid + NAD ↔ 2-Ketobutyric acid + Hydrogen Ion + NADH
2-Ketobutyric acid + Coenzyme A + NAD → NADH + Propionyl-CoA
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH
S-Methylmalonyl-CoA → R-Methylmalonyl-CoA
Malonyl-CoA → Acetyl-CoA + Carbon dioxide
Acetyl-CoA + Adenosine triphosphate + Hydrogen carbonate → Adenosine diphosphate + Malonyl-CoA + Phosphoric acid
3-Hydroxypropionyl-CoA ↔ Acrylyl-CoA + Water
Adenosine monophosphate + Propionyl-CoA + Pyrophosphate ↔ Adenosine triphosphate + Pantetheine + Propinol adenylate
Adenosine triphosphate + Pantetheine + Propinol adenylate ↔ Adenosine monophosphate + Propionyl-CoA + Pyrophosphate
3-Hydroxypropionyl-CoA + Water ↔ Coenzyme A + Hydroxypropionic acid
Acrylyl-CoA + electron-transfer flavoprotein ↔ Propionyl-CoA + Reduced electron-transfer flavoprotein
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD + Water → Hydrogen carbonate + NADH + Propionyl-CoA
Acetyl-CoA acetyltransferase, mitochondrial
Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Hydrogen + S-Methylmalonyl-CoA
Propinol adenylate ↔ Adenosine monophosphate + Propionic acid