Isovaleryl-CoA (→) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Blood Vessel
3-hydroxyisobutyrate dehydrogenase, mitochondrial
Isobutyryl-CoA + electron-transfer flavoprotein → Methacrylyl-CoA + Reduced electron-transfer flavoprotein
3-Methyl-2-oxovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
Coenzyme A + S-(2-Methylbutanoyl)-dihydrolipoamide → 2-Methylbutyryl-CoA + Dihydrolipoamide
Isovaleryl-CoA dehydrogenase, mitochondrial
2 Acetyl-CoA → Acetoacetyl-CoA + Coenzyme A
Acetoacetic acid + Succinyl-CoA → Acetoacetyl-CoA + Succinic acid
Methylmalonic acid → R-Methylmalonyl-CoA
Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
2-Methyl-1-hydroxypropyl-ThPP + 3-Methyl-1-hydroxybutyl-ThPP → Thiamine pyrophosphate
Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
Isovaleryl-CoA + electron-transfer flavoprotein → 3-Methylcrotonyl-CoA + Reduced electron-transfer flavoprotein
Coenzyme A + S-(3-Methylbutanoyl)-dihydrolipoamide-E → Dihydrolipoamide + Isovaleryl-CoA
3-Methyl-1-hydroxybutyl-ThPP + Lipoamide → S-(3-Methylbutanoyl)-dihydrolipoamide-E
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic acid
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
3-Methylcrotonyl-CoA + Adenosine triphosphate + Hydrogen carbonate → 3-Methylglutaconyl-CoA + Adenosine diphosphate
Isovaleryl-CoA + electron-transfer flavoprotein ↔ 3-Methylcrotonyl-CoA + Reduced electron-transfer flavoprotein
3-Hydroxyisovaleryl-CoA → 3-Methylcrotonyl-CoA + Water
3-Methylglutaconyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
2-Methyl-1-hydroxypropyl-ThPP → S-(2-Methylpropionyl)-dihydrolipoamide-E
L-Valine + Oxoglutaric acid → L-Glutamic acid + α-Ketoisovaleric acid
3-Hydroxy-3-methylglutaryl-CoA → Acetyl-CoA
3-Hydroxy-3-methylglutaryl-CoA → Acetoacetic acid
Hydroxymethylglutaryl-CoA lyase, mitochondrial
4-aminobutyrate aminotransferase, mitochondrial
Dihydrolipoyl dehydrogenase, mitochondrial
Propionyl-CoA carboxylase beta chain, mitochondrial
Hydroxymethylglutaryl-CoA synthase, mitochondrial
Acetyl-CoA acetyltransferase, mitochondrial
Isobutyryl-CoA dehydrogenase, mitochondrial
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD + Water → Hydrogen carbonate + NADH + Propionyl-CoA
Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Phosphate + S-Methylmalonyl-CoA
(S)-Methylmalonic acid semialdehyde + Oxygen + Water → Hydrogen peroxide + Methylmalonic acid
(S)-3-Hydroxyisobutyryl-CoA + Water → (S)-3-Hydroxyisobutyric acid + Coenzyme A
Isobutyryl-CoA + electron-transfer flavoprotein ↔ Methacrylyl-CoA + Reduced electron-transfer flavoprotein
Methacrylyl-CoA + Water → (S)-3-Hydroxyisobutyryl-CoA
Coenzyme A + S-(2-Methylpropionyl)-dihydrolipoamide-E → Dihydrolipoamide + Isobutyryl-CoA
Lipoamide → S-(2-Methylpropionyl)-dihydrolipoamide-E
Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
2-Methylacetoacetyl-CoA + Coenzyme A → Acetyl-CoA + Propionyl-CoA
Tiglyl-CoA + Water → 2-Methyl-3-hydroxybutyryl-CoA
2-Methyl-3-hydroxybutyryl-CoA + NAD ↔ 2-Methylacetoacetyl-CoA + NADH
2-Methylbutyryl-CoA + electron-transfer flavoprotein → Reduced electron-transfer flavoprotein + Tiglyl-CoA
2-Methylbutyryl-CoA + electron-transfer flavoprotein ↔ Reduced electron-transfer flavoprotein + Tiglyl-CoA
S-Methylmalonyl-CoA → R-Methylmalonyl-CoA
Branched-chain-amino-acid aminotransferase, cytosolic
Methylglutaconyl-CoA hydratase, mitochondrial
2-Methyl-1-hydroxypropyl-ThPP + Lipoamide → S-(2-Methylbutanoyl)-dihydrolipoamide + Thiamine pyrophosphate
L-Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + L-Glutamic acid
(S)-beta-Aminoisobutyric acid + Oxoglutaric acid ↔ (S)-Methylmalonic acid semialdehyde + L-Glutamic acid
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Methylmalonyl-CoA epimerase, mitochondrial
Propionyl-CoA carboxylase alpha chain, mitochondrial
Thiamine pyrophosphate + α-Ketoisovaleric acid → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
Ketoleucine + Thiamine pyrophosphate ? 3-Methyl-1-hydroxybutyl-ThPP + Carbon dioxide
(S)-3-Hydroxyisobutyric acid + NAD → (S)-Methylmalonic acid semialdehyde + Hydrogen Ion + NADH
Dihydrolipoamide + NAD → Hydrogen Ion + Lipoamide + NADH
Acetoacetyl-CoA + Acetyl-CoA + Water → 3-Hydroxy-3-methylglutaryl-CoA + Coenzyme A