Pathway Details

Recommended name

Valine, leucine and isoleucine degradation

Alternative names

Reactions

ATP + L-Isoleucine + tRNA(Ile) <-> Diphosphate + AMP + L-Isoleucyl-tRNA(Ile)

ATP + L-Valine + tRNA(Val) <-> Diphosphate + AMP + L-Valyl-tRNA(Val)

2 Acetyl-CoA <-> Coenzyme A + Acetoacetyl-CoA

Protein <-> L-Leucyl-tRNA(Leu)

Protein <-> L-Valyl-tRNA(Val)

Protein <-> L-Isoleucyl-tRNA(Ile)

Succinyl-CoA + Acetoacetate <-> Succinate + Acetoacetyl-CoA

(R)-2-Methyl-3-oxopropanoyl-CoA <-> Succinyl-CoA

NAD+ + Coenzyme A + 2-Methyl-3-oxopropanoate <-> NADH + H+ + CO2 + Propanoyl-CoA

Acetyl-CoA + Propanoyl-CoA <-> Coenzyme A + 2-Methylacetoacetyl-CoA

NAD+ + Coenzyme A + (S)-Methylmalonate semialdehyde <-> NADH + H+ + CO2 + Propanoyl-CoA

NAD+ + H2O + L-Leucine <-> NADH + H+ + NH3 + 4-Methyl-2-oxopentanoate

L-Leucine + 2-Oxoglutarate <-> L-Glutamate + 4-Methyl-2-oxopentanoate

L-Leucine <-> (3R)-beta-Leucine

NAD+ + Coenzyme A + alpha-Ketovaline <-> NADH + H+ + CO2 + 2-Methylpropanoyl-CoA

L-Valine + 2-Oxoglutarate <-> L-Glutamate + alpha-Ketovaline

Pyruvate + L-Valine <-> L-Alanine + alpha-Ketovaline

ATP + Coenzyme A + Acetoacetate <-> Diphosphate + AMP + Acetoacetyl-CoA

3-Hydroxy-3-methylglutaryl-CoA <-> Acetyl-CoA + Acetoacetate

NAD+ + H2O + L-Valine <-> NADH + H+ + NH3 + alpha-Ketovaline

L-Valine <-> (S)-Methylmalonate semialdehyde

NAD+ + Coenzyme A + 4-Methyl-2-oxopentanoate <-> NADH + H+ + CO2 + 3-Methylbutanoyl-CoA

CO2 + 4-Methyl-2-oxopentanoate <-> 3-Carboxy-4-methyl-2-oxopentanoate

Lipoamide + alpha-Ketovaline <-> CO2 + S-(2-Methylpropanoyl)-dihydrolipoamide

Lipoamide + 4-Methyl-2-oxopentanoate <-> CO2 + S-(3-Methylbutanoyl)-dihydrolipoamide

ATP + Hydrogencarbonate + Propanoyl-CoA <-> ADP + Phosphate + (S)-2-Methyl-3-oxopropanoyl-CoA

Coenzyme A + 3-Hydroxy-3-methylglutaryl-CoA <-> H2O + Acetyl-CoA + Acetoacetyl-CoA

Pyruvate + (R)-3-Amino-2-methylpropanoate <-> L-Alanine + 2-Methyl-3-oxopropanoate

3-Hydroxy-3-methylglutaryl-CoA <-> H2O + trans-3-Methylglutaconyl-CoA

NAD+ + H2O + L-Isoleucine <-> NADH + H+ + NH3 + (R)-2-Oxo-3-methylpentanoate

H2O + L-Isoleucine + O2 <-> NH3 + H2O2 + 3-Methyl-2-oxopentanoate

L-Isoleucine + 2-Oxoglutarate <-> L-Glutamate + 3-Methyl-2-oxopentanoate

L-Isoleucine + 2-Oxoglutarate <-> L-Glutamate + (R)-2-Oxo-3-methylpentanoate

L-Valine + (S)-3-Methyl-2-oxopentanoate <-> L-Isoleucine + alpha-Ketovaline

O2 + 2-Methylpropanoyl-CoA <-> 2 H2O + 2-Methylprop-2-enoyl-CoA

FAD + 2-Methylpropanoyl-CoA <-> FADH2 + 2-Methylprop-2-enoyl-CoA

Dihydrolipoamide + 2-Methylpropanoyl-CoA <-> Coenzyme A + S-(2-Methylpropanoyl)-dihydrolipoamide

2-Methylpropanoyl-CoA <-> Branched chain fatty acid

H2O + (S)-2-Methyl-3-oxopropanoyl-CoA <-> Coenzyme A + Methylmalonate

(R)-2-Methyl-3-oxopropanoyl-CoA <-> (S)-2-Methyl-3-oxopropanoyl-CoA

O2 + 2-Methylbutanoyl-CoA <-> 2 H2O + 2-Methylbut-2-enoyl-CoA

NAD+ + Coenzyme A + 3-Methyl-2-oxopentanoate <-> NADH + H+ + CO2 + 2-Methylbutanoyl-CoA

FAD + 2-Methylbutanoyl-CoA <-> FADH2 + 2-Methylbut-2-enoyl-CoA

Dihydrolipoamide + 2-Methylbutanoyl-CoA <-> Coenzyme A + S-(2-Methylbutanoyl)-dihydrolipoamide

2-Methylbutanoyl-CoA <-> Branched chain fatty acid

H2O + (R)-2-Methyl-3-oxopropanoyl-CoA <-> Coenzyme A + Methylmalonate

NAD+ + H2O + (S)-Methylmalonate semialdehyde <-> NADH + H+ + Methylmalonate

Methylmalonate + O2.- <-> H2O + O2 + (S)-Methylmalonate semialdehyde

NAD+ + H2O + (3R)-beta-Leucine <-> NADH + H+ + NH3 + 3-Oxo-4-methylpentanoate

2-Isopropylmalate <-> H2O + 2-Isopropylmaleate

3-Isopropylmalate <-> H2O + 2-Isopropylmaleate

FAD + 3-Methylbutanoyl-CoA <-> FADH2 + 3-Methylcrotonyl-CoA

Dihydrolipoamide + 3-Methylbutanoyl-CoA <-> Coenzyme A + S-(3-Methylbutanoyl)-dihydrolipoamide

3-Methylbutanoyl-CoA <-> Branched chain fatty acid

3-Hydroxyisovaleryl-CoA <-> H2O + 3-Methylcrotonyl-CoA

ATP + Hydrogencarbonate + 3-Methylcrotonyl-CoA <-> ADP + Phosphate + trans-3-Methylglutaconyl-CoA

L-Alanine + (S)-Methylmalonate semialdehyde <-> Pyruvate + L-3-Amino-isobutanoate

2-Oxoglutarate + L-3-Amino-isobutanoate <-> L-Glutamate + (S)-Methylmalonate semialdehyde

NAD+ + (2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA <-> NADH + H+ + 2-Methylacetoacetyl-CoA

(2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA <-> H2O + 2-Methylbut-2-enoyl-CoA

H2O + 2-Methylprop-2-enoyl-CoA <-> (S)-3-Hydroxyisobutyryl-CoA

Lipoamide + 3-Methyl-2-oxopentanoate <-> CO2 + S-(2-Methylbutanoyl)-dihydrolipoamide

NAD+ + 3-Isopropylmalate <-> NADH + H+ + 3-Carboxy-4-methyl-2-oxopentanoate

NADP+ + (R)-2,3-Dihydroxy-3-methylbutanoate <-> H+ + NADPH + (R)-3-Hydroxy-3-methyl-2-oxobutanoate

(R)-2,3-Dihydroxy-3-methylbutanoate <-> H2O + alpha-Ketovaline

Thiamine diphosphate + (S)-2-Acetolactate <-> Pyruvate + 2-(alpha-Hydroxyethyl)thiamine diphosphate

2-(alpha-Hydroxyethyl)thiamine diphosphate + 2-Oxobutyrate <-> Thiamine diphosphate + (S)-2-Aceto-2-hydroxybutanoate

H2O + (S)-3-Hydroxyisobutyryl-CoA <-> Coenzyme A + (S)-3-Hydroxyisobutyrate

NAD+ + (S)-3-Hydroxyisobutyrate <-> NADH + H+ + (S)-Methylmalonate semialdehyde

3-Oxo-4-methylpentanoate <-> Acetate + (R)-3-Methyl-2-oxobutanoate

NADP+ + (R)-2,3-Dihydroxy-3-methylpentanoate <-> H+ + NADPH + (S)-2-Hydroxy-3-methyl-3-oxopentanoate

(S)-2-Aceto-2-hydroxybutanoate <-> (S)-2-Hydroxy-3-methyl-3-oxopentanoate

(R)-2,3-Dihydroxy-3-methylpentanoate <-> H2O + (R)-2-Oxo-3-methylpentanoate

(S)-2-Acetolactate <-> (R)-3-Hydroxy-3-methyl-2-oxobutanoate

CO2 + 2-(alpha-Hydroxyethyl)thiamine diphosphate <-> Pyruvate + Thiamine diphosphate

Riboflavin-5-phosphate + 2-Hydroxyisocaproate <-> 4-Methyl-2-oxopentanoate + Reduced FMN

L-Leucine + alpha-Ketovaline <-> L-Valine + 4-Methyl-2-oxopentanoate

L-Valine + alpha-Ketovaline <-> L-Valine + alpha-Ketovaline

L-Isoleucine + 2-Oxoglutarate <-> L-Glutamate + (S)-3-Methyl-2-oxopentanoate

O2 + (S)-2-Hydroxyisovalerate <-> H2O2 + alpha-Ketovaline

H2O + L-Leucine + O2 <-> NH3 + H2O2 + 4-Methyl-2-oxopentanoate

H2O + 2-Methylmalonyl-CoA <-> Coenzyme A + Methylmalonate

Classification

Amino Acid Metabolism