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Valine, leucine and isoleucine degradation

State Icon Reaction EC-Number Confidence maximal confidence score from EnzymeDetector
NAD+ + (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA <=> H+ + NADH + 2-methylacetoacetyl-CoA 1.1.1.178 3-hydroxy-2-methylbutyryl-CoA dehydrogenase
1.1.1.35 3-hydroxyacyl-CoA dehydrogenase


methylmalonate + H2O2 <=> L-2-methyl-3-oxopropionate + O2 + H2O 1.2.3.1 aldehyde oxidase

enzyme_N6-(dihydrolipoyl)lysine + NAD+ <=> [dihydrolipoyllysine-residue(2-methylpropanoyl)transferase]-lipoyllysine + NADH + H+ 1.8.1.4 dihydrolipoyl dehydrogenase

(S)-3-methyl-2-oxopentanoate + thiamine_diphosphate <=> 2-Methyl-1-hydroxybutyl-ThPP + CO2 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

CoA + acetoacetate + ATP <=> acetoacetyl-CoA + diphosphate + AMP 6.2.1.16 acetoacetate---CoA ligase

(R)-2-methylmalonyl-CoA <=> succinyl-CoA 5.4.99.2 methylmalonyl-CoA mutase
5.4.99.63 ethylmalonyl-CoA mutase
5.4.99.64 2-hydroxyisobutanoyl-CoA mutase



L-2-methyl-3-oxopropionate + NAD+ + H2O <=> methylmalonate + NADH + H+ 1.2.1.3 aldehyde dehydrogenase (NAD+)

(S)-3-hydroxy-3-methylglutaryl-CoA <=> acetoacetate + acetyl-CoA 4.1.3.4 hydroxymethylglutaryl-CoA lyase

3-hydroxyisovaleryl-CoA <=> 3-Hydroxy-3-methylbutyrate not assigned

succinyl-CoA + acetoacetate <=> succinate + acetoacetyl-CoA 2.8.3.18 succinyl-CoA:acetate CoA-transferase
2.8.3.5 3-oxoacid CoA-transferase


L-leucine + NAD+ + H2O <=> H+ + NH3 + NADH + 4-methyl-2-oxopentanoate 1.4.1.1 alanine dehydrogenase
1.4.1.20 phenylalanine dehydrogenase
1.4.1.23 valine dehydrogenase (NAD+)
1.4.1.8 valine dehydrogenase (NADP+)
1.4.1.9 leucine dehydrogenase





acetoacetyl-CoA + H2O + acetyl-CoA <=> H+ + CoA + (S)-3-hydroxy-3-methylglutaryl-CoA 2.3.3.10 hydroxymethylglutaryl-CoA synthase

3-Methyl-1-hydroxybutyl-ThPP + Enzyme_N6-(lipoyl)lysine <=> [dihydrolipoyllysine-residue_(2-methylpropanoyl)transferase]_S-(3-methylbutanoyl)dihydrolipoyllysine + thiamine_diphosphate 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

2-methylacetoacetyl-CoA + CoA <=> propionyl-CoA + acetyl-CoA 2.3.1.16 acetyl-CoA C-acyltransferase
2.3.1.9 acetyl-CoA C-acetyltransferase


L-leucine + 2-oxoglutarate <=> L-glutamate + 4-methyl-2-oxopentanoate 2.6.1.1 aspartate transaminase
2.6.1.2 alanine transaminase
2.6.1.39 2-aminoadipate transaminase
2.6.1.42 branched-chain-amino-acid transaminase
2.6.1.57 aromatic-amino-acid transaminase
2.6.1.6 leucine transaminase
2.6.1.67 2-aminohexanoate transaminase
2.6.1.9 histidinol-phosphate transaminase








L-valine + 2-oxoglutarate <=> L-glutamate + 2-oxoisovalerate 2.6.1.39 2-aminoadipate transaminase
2.6.1.42 branched-chain-amino-acid transaminase
2.6.1.6 leucine transaminase
2.6.1.66 valine---pyruvate transaminase




(2S,3S)-3-hydroxy-2-methylbutanoyl-CoA <=> tigloyl-CoA + H2O 4.2.1.17 enoyl-CoA hydratase

L-isoleucine + 2-oxoglutarate <=> L-glutamate + (S)-3-methyl-2-oxopentanoate 2.6.1.42 branched-chain-amino-acid transaminase
2.6.1.67 2-aminohexanoate transaminase


(S)-2-methylbutanoyl-CoA + enzyme_N6-(dihydrolipoyl)lysine <=> CoA + [dihydrolipoyllysine-residue_(2-methylpropanoyl)transferase]_S-(2-methylbutanoyl)dihydrolipoyllysine 2.3.1.168 dihydrolipoyllysine-residue (2-methylpropanoyl)transferase

H2O + (S)-methylmalonyl-CoA <=> H+ + CoA + methylmalonate 3.1.2.17 (S)-methylmalonyl-CoA hydrolase
3.1.2.20 acyl-CoA hydrolase
3.1.2.4 3-hydroxyisobutyryl-CoA hydrolase



3-hydroxyisovaleryl-CoA <=> 3-methylcrotonoyl-CoA + H2O 4.2.1.17 enoyl-CoA hydratase

4-methyl-2-oxopentanoate + thiamine_diphosphate <=> 3-Methyl-1-hydroxybutyl-ThPP + CO2 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

2-Methyl-1-hydroxypropyl-ThPP + Enzyme_N6-(lipoyl)lysine <=> [dihydrolipoyllysine-residue_(2-methylpropanoyl)transferase]_S-(2-methylpropanoyl)dihydrolipoyllysine + thiamine_diphosphate 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

2-Methyl-1-hydroxybutyl-ThPP + Enzyme_N6-(lipoyl)lysine <=> [dihydrolipoyllysine-residue_(2-methylpropanoyl)transferase]_S-(2-methylbutanoyl)dihydrolipoyllysine + thiamine_diphosphate 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

(S)-2-methylbutanoyl-CoA + acceptor <=> tigloyl-CoA + reduced_acceptor 1.3.8.1 short-chain acyl-CoA dehydrogenase
1.3.8.7 medium-chain acyl-CoA dehydrogenase
1.3.99.12 2-methylacyl-CoA dehydrogenase



2-oxoisovalerate + CoA + oxidized_ferredoxin <=> isobutyryl-CoA + CO2 + reduced_ferredoxin_[iron-sulfur]_cluster + H+ 1.2.7.7 3-methyl-2-oxobutanoate dehydrogenase (ferredoxin)

isobutyryl-CoA + acceptor <=> methacryloyl-CoA + reduced_acceptor 1.3.8.1 short-chain acyl-CoA dehydrogenase
1.3.8.7 medium-chain acyl-CoA dehydrogenase
1.3.99
1.3.99.12 2-methylacyl-CoA dehydrogenase




3-methylcrotonoyl-CoA + HCO3- + ATP <=> trans-3-methylglutaconyl-CoA + ADP + phosphate + H+ 6.4.1.4 methylcrotonoyl-CoA carboxylase
6.4.1.5 geranoyl-CoA carboxylase


(S)-3-methyl-2-oxopentanoate + CoA + oxidized_ferredoxin <=> (S)-2-methylbutanoyl-CoA + CO2 + reduced_ferredoxin_[iron-sulfur]_cluster + H+ 1.2.7.7 3-methyl-2-oxobutanoate dehydrogenase (ferredoxin)

(S)-3-hydroxy-2-methylpropanoyl-CoA <=> methacryloyl-CoA + H2O 4.2.1.17 enoyl-CoA hydratase

L-3-hydroxyisobutyrate + NAD+ <=> H+ + NADH + L-2-methyl-3-oxopropionate 1.1.1.291 2-hydroxymethylglutarate dehydrogenase
1.1.1.31 3-hydroxyisobutyrate dehydrogenase
1.1.1.35 3-hydroxyacyl-CoA dehydrogenase



(S)-3-hydroxy-2-methylpropanoyl-CoA + H2O <=> L-3-hydroxyisobutyrate + H+ + CoA 3.1.2.4 3-hydroxyisobutyryl-CoA hydrolase

isovaleryl-CoA + enzyme_N6-(dihydrolipoyl)lysine <=> CoA + [dihydrolipoyllysine-residue_(2-methylpropanoyl)transferase]_S-(3-methylbutanoyl)dihydrolipoyllysine 2.3.1.168 dihydrolipoyllysine-residue (2-methylpropanoyl)transferase

acetyl-CoA <=> acetoacetyl-CoA + CoA 1.1.1.34 hydroxymethylglutaryl-CoA reductase (NADPH)
2.3.1.155 acetyl-CoA C-myristoyltransferase
2.3.1.16 acetyl-CoA C-acyltransferase
2.3.1.38 [acyl-carrier-protein] S-acetyltransferase
2.3.1.9 acetyl-CoA C-acetyltransferase





isovaleryl-CoA <=> Branched_chain_fatty_acid not assigned

L-2-methyl-3-oxopropionate + L-alanine <=> (S)-3-amino-2-methylpropanoate + pyruvate 2.6.1
2.6.1.18 beta-alanine---pyruvate transaminase


methylmalonate + CoA + ATP <=> (R)-2-methylmalonyl-CoA + AMP + diphosphate 6.2.1

(R)-2-methylmalonyl-CoA <=> (S)-methylmalonyl-CoA 5.1.99.1 methylmalonyl-CoA epimerase

L-isoleucine + H2O + O2 <=> (S)-3-methyl-2-oxopentanoate + NH3 + H2O2 1.4.3.2 L-amino-acid oxidase

4-methyl-2-oxopentanoate + CoA + oxidized_ferredoxin <=> isovaleryl-CoA + CO2 + reduced_ferredoxin_[iron-sulfur]_cluster + H+ 1.2.7.7 3-methyl-2-oxobutanoate dehydrogenase (ferredoxin)
1.2.7.8 indolepyruvate ferredoxin oxidoreductase


(S)-3-hydroxy-3-methylglutaryl-CoA <=> H2O + trans-3-methylglutaconyl-CoA 4.2.1.18 methylglutaconyl-CoA hydratase

2-Methyl-3-oxopropanoate + CoA + NAD+ <=> propionyl-CoA + CO2 + NADH 1.2.1.18 malonate-semialdehyde dehydrogenase (acetylating)
1.2.1.27 methylmalonate-semialdehyde dehydrogenase (CoA-acylating)


2-oxoisovalerate + thiamine_diphosphate <=> 2-Methyl-1-hydroxypropyl-ThPP + CO2 1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)

L-valine + H2O + NAD+ <=> 2-oxoisovalerate + NH3 + NADH + H+ 1.4.1.1 alanine dehydrogenase
1.4.1.2 glutamate dehydrogenase
1.4.1.20 phenylalanine dehydrogenase
1.4.1.23 valine dehydrogenase (NAD+)
1.4.1.5 L-amino-acid dehydrogenase
1.4.1.8 valine dehydrogenase (NADP+)
1.4.1.9 leucine dehydrogenase







isobutyryl-CoA <=> Branched_chain_fatty_acid not assigned

isovaleryl-CoA + FAD <=> 3-methylcrotonoyl-CoA + FADH2 1.3.8.4 isovaleryl-CoA dehydrogenase
1.3.8.7 medium-chain acyl-CoA dehydrogenase


(R)-3-Amino-2-methylpropanoate + pyruvate <=> 2-Methyl-3-oxopropanoate + L-alanine 2.6.1.40 (R)-3-amino-2-methylpropionate---pyruvate transaminase

ATP + propionyl-CoA + HCO3- <=> H+ + (S)-methylmalonyl-CoA + phosphate + ADP 6.4.1.2 acetyl-CoA carboxylase
6.4.1.3 propionyl-CoA carboxylase


2-oxoglutarate + (S)-3-amino-2-methylpropanoate <=> L-glutamate + L-2-methyl-3-oxopropionate 2.6.1.22 (S)-3-amino-2-methylpropionate transaminase

isobutyryl-CoA + enzyme_N6-(dihydrolipoyl)lysine <=> CoA + enzyme_N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine 2.3.1.168 dihydrolipoyllysine-residue (2-methylpropanoyl)transferase

(S)-2-methylbutanoyl-CoA <=> Branched_chain_fatty_acid not assigned

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