Pathway Details

 Recommended name Valine, leucine and isoleucine degradation
 Alternative names
 Reactions
L-Leucine <-> (3R)-beta-Leucine
ATP + Hydrogencarbonate + Propanoyl-CoA <-> ADP + Phosphate + (S)-2-Methyl-3-oxopropanoyl-CoA
H2O + 2-Methylprop-2-enoyl-CoA <-> (S)-3-Hydroxyisobutyryl-CoA
Pyruvate + L-Valine <-> L-Alanine + alpha-Ketovaline
H2O + (R)-2-Methyl-3-oxopropanoyl-CoA <-> Coenzyme A + Methylmalonate
2 Acetyl-CoA <-> Coenzyme A + Acetoacetyl-CoA
2-Isopropylmalate <-> H2O + 2-Isopropylmaleate
L-Isoleucine + 2-Oxoglutarate <-> L-Glutamate + (R)-2-Oxo-3-methylpentanoate
Acetyl-CoA + Propanoyl-CoA <-> Coenzyme A + 2-Methylacetoacetyl-CoA
NAD+ + Coenzyme A + (S)-Methylmalonate semialdehyde <-> NADH + H+ + CO2 + Propanoyl-CoA
3-Isopropylmalate <-> H2O + 2-Isopropylmaleate
(S)-2-Acetolactate <-> (R)-3-Hydroxy-3-methyl-2-oxobutanoate
Protein <-> L-Isoleucyl-tRNA(Ile)
Lipoamide + 3-Methyl-2-oxopentanoate <-> CO2 + S-(2-Methylbutanoyl)-dihydrolipoamide
Dihydrolipoamide + 2-Methylbutanoyl-CoA <-> Coenzyme A + S-(2-Methylbutanoyl)-dihydrolipoamide
2-Methylbutanoyl-CoA <-> Branched chain fatty acid
FAD + 3-Methylbutanoyl-CoA <-> FADH2 + 3-Methylcrotonyl-CoA
ATP + Hydrogencarbonate + 3-Methylcrotonyl-CoA <-> ADP + Phosphate + trans-3-Methylglutaconyl-CoA
NAD+ + H2O + (S)-Methylmalonate semialdehyde <-> NADH + H+ + Methylmalonate
FAD + 2-Methylbutanoyl-CoA <-> FADH2 + 2-Methylbut-2-enoyl-CoA
L-Valine <-> (S)-Methylmalonate semialdehyde
L-Isoleucine + 2-Oxoglutarate <-> L-Glutamate + 3-Methyl-2-oxopentanoate
L-Isoleucine + 2-Oxoglutarate <-> L-Glutamate + (S)-3-Methyl-2-oxopentanoate
Succinyl-CoA + Acetoacetate <-> Succinate + Acetoacetyl-CoA
NAD+ + H2O + L-Valine <-> NADH + H+ + NH3 + alpha-Ketovaline
Lipoamide + 4-Methyl-2-oxopentanoate <-> CO2 + S-(3-Methylbutanoyl)-dihydrolipoamide
NAD+ + H2O + L-Leucine <-> NADH + H+ + NH3 + 4-Methyl-2-oxopentanoate
NAD+ + 3-Isopropylmalate <-> NADH + H+ + 3-Carboxy-4-methyl-2-oxopentanoate
ATP + L-Valine + tRNA(Val) <-> Diphosphate + AMP + L-Valyl-tRNA(Val)
NAD+ + Coenzyme A + 3-Methyl-2-oxopentanoate <-> NADH + H+ + CO2 + 2-Methylbutanoyl-CoA
NAD+ + Coenzyme A + alpha-Ketovaline <-> NADH + H+ + CO2 + 2-Methylpropanoyl-CoA
L-Valine + alpha-Ketovaline <-> L-Valine + alpha-Ketovaline
CO2 + 2-(alpha-Hydroxyethyl)thiamine diphosphate <-> Pyruvate + Thiamine diphosphate
3-Hydroxyisovaleryl-CoA <-> H2O + 3-Methylcrotonyl-CoA
O2 + 2-Methylbutanoyl-CoA <-> 2 H2O + 2-Methylbut-2-enoyl-CoA
2-Oxoglutarate + L-3-Amino-isobutanoate <-> L-Glutamate + (S)-Methylmalonate semialdehyde
L-Alanine + (S)-Methylmalonate semialdehyde <-> Pyruvate + L-3-Amino-isobutanoate
NAD+ + (S)-3-Hydroxyisobutyrate <-> NADH + H+ + (S)-Methylmalonate semialdehyde
3-Oxo-4-methylpentanoate <-> Acetate + (R)-3-Methyl-2-oxobutanoate
Protein <-> L-Leucyl-tRNA(Leu)
Coenzyme A + 3-Hydroxy-3-methylglutaryl-CoA <-> H2O + Acetyl-CoA + Acetoacetyl-CoA
Lipoamide + alpha-Ketovaline <-> CO2 + S-(2-Methylpropanoyl)-dihydrolipoamide
L-Valine + (S)-3-Methyl-2-oxopentanoate <-> L-Isoleucine + alpha-Ketovaline
L-Leucine + 2-Oxoglutarate <-> L-Glutamate + 4-Methyl-2-oxopentanoate
NAD+ + (2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA <-> NADH + H+ + 2-Methylacetoacetyl-CoA
FAD + 2-Methylpropanoyl-CoA <-> FADH2 + 2-Methylprop-2-enoyl-CoA
(R)-2,3-Dihydroxy-3-methylpentanoate <-> H2O + (R)-2-Oxo-3-methylpentanoate
3-Hydroxy-3-methylglutaryl-CoA <-> Acetyl-CoA + Acetoacetate
NAD+ + Coenzyme A + 4-Methyl-2-oxopentanoate <-> NADH + H+ + CO2 + 3-Methylbutanoyl-CoA
NAD+ + H2O + (3R)-beta-Leucine <-> NADH + H+ + NH3 + 3-Oxo-4-methylpentanoate
NAD+ + H2O + L-Isoleucine <-> NADH + H+ + NH3 + (R)-2-Oxo-3-methylpentanoate
H2O + L-Leucine + O2 <-> NH3 + H2O2 + 4-Methyl-2-oxopentanoate
L-Valine + 2-Oxoglutarate <-> L-Glutamate + alpha-Ketovaline
(S)-2-Aceto-2-hydroxybutanoate <-> (S)-2-Hydroxy-3-methyl-3-oxopentanoate
Protein <-> L-Valyl-tRNA(Val)
(R)-2,3-Dihydroxy-3-methylbutanoate <-> H2O + alpha-Ketovaline
H2O + (S)-3-Hydroxyisobutyryl-CoA <-> Coenzyme A + (S)-3-Hydroxyisobutyrate
O2 + 2-Methylpropanoyl-CoA <-> 2 H2O + 2-Methylprop-2-enoyl-CoA
O2 + (S)-2-Hydroxyisovalerate <-> H2O2 + alpha-Ketovaline
2-Methylpropanoyl-CoA <-> Branched chain fatty acid
(R)-2-Methyl-3-oxopropanoyl-CoA <-> Succinyl-CoA
3-Hydroxy-3-methylglutaryl-CoA <-> H2O + trans-3-Methylglutaconyl-CoA
ATP + L-Isoleucine + tRNA(Ile) <-> Diphosphate + AMP + L-Isoleucyl-tRNA(Ile)
3-Methylbutanoyl-CoA <-> Branched chain fatty acid
H2O + L-Isoleucine + O2 <-> NH3 + H2O2 + 3-Methyl-2-oxopentanoate
Thiamine diphosphate + (S)-2-Acetolactate <-> Pyruvate + 2-(alpha-Hydroxyethyl)thiamine diphosphate
(2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA <-> H2O + 2-Methylbut-2-enoyl-CoA
(R)-2-Methyl-3-oxopropanoyl-CoA <-> (S)-2-Methyl-3-oxopropanoyl-CoA
Dihydrolipoamide + 3-Methylbutanoyl-CoA <-> Coenzyme A + S-(3-Methylbutanoyl)-dihydrolipoamide
Dihydrolipoamide + 2-Methylpropanoyl-CoA <-> Coenzyme A + S-(2-Methylpropanoyl)-dihydrolipoamide
CO2 + 4-Methyl-2-oxopentanoate <-> 3-Carboxy-4-methyl-2-oxopentanoate
L-Leucine + alpha-Ketovaline <-> L-Valine + 4-Methyl-2-oxopentanoate
Riboflavin-5-phosphate + 2-Hydroxyisocaproate <-> 4-Methyl-2-oxopentanoate + Reduced FMN
NADP+ + (R)-2,3-Dihydroxy-3-methylbutanoate <-> H+ + NADPH + (R)-3-Hydroxy-3-methyl-2-oxobutanoate
Methylmalonate + O2.- <-> H2O + O2 + (S)-Methylmalonate semialdehyde
2-(alpha-Hydroxyethyl)thiamine diphosphate + 2-Oxobutyrate <-> Thiamine diphosphate + (S)-2-Aceto-2-hydroxybutanoate
NADP+ + (R)-2,3-Dihydroxy-3-methylpentanoate <-> H+ + NADPH + (S)-2-Hydroxy-3-methyl-3-oxopentanoate
 Classification
Amino Acid Metabolism
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