KEGG   PATHWAY: hsa00280
Entry
hsa00280                    Pathway                                
Name
Valine, leucine and isoleucine degradation - Homo sapiens (human)
Class
Metabolism; Amino acid metabolism
Pathway map
hsa00280  Valine, leucine and isoleucine degradation
hsa00280

Module
hsa_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA [PATH:hsa00280]
hsa_M00741  Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA [PATH:hsa00280]
Network
nt06024  Valine, leucine and isoleucine degradation
nt06032  Lipoic acid metabolism
  Element
N00832  Branched-chain amino acids degradation 1
N00842  Propanoyl-CoA metabolism
N00851  Leucine degradation
N00852  Valine degradation
N00856  Isoleucine degradation
N01619  Branched-chain amino acids degradation 2
Other DBs
GO: 0006550 0006552 0006574
Organism
Homo sapiens (human) [GN:hsa]
Gene
10449  ACAA2; acetyl-CoA acyltransferase 2 [KO:K07508] [EC:2.3.1.16]
11112  HIBADH; 3-hydroxyisobutyrate dehydrogenase [KO:K00020] [EC:1.1.1.31]
1629  DBT; dihydrolipoamide branched chain transacylase E2 [KO:K09699] [EC:2.3.1.168]
1738  DLD; dihydrolipoamide dehydrogenase [KO:K00382] [EC:1.8.1.4]
18  ABAT; 4-aminobutyrate aminotransferase [KO:K13524] [EC:2.6.1.19 2.6.1.22]
1892  ECHS1; enoyl-CoA hydratase, short chain 1 [KO:K07511] [EC:4.2.1.17]
1962  EHHADH; enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
197322  ACSF3; acyl-CoA synthetase family member 3 [KO:K18660] [EC:6.2.1.76 6.2.1.-]
217  ALDH2; aldehyde dehydrogenase 2 family member [KO:K00128] [EC:1.2.1.3]
219  ALDH1B1; aldehyde dehydrogenase 1 family member B1 [KO:K00128] [EC:1.2.1.3]
223  ALDH9A1; aldehyde dehydrogenase 9 family member A1 [KO:K00149] [EC:1.2.1.47 1.2.1.3]
224  ALDH3A2; aldehyde dehydrogenase 3 family member A2 [KO:K00128] [EC:1.2.1.3]
259307  IL4I1; interleukin 4 induced 1 [KO:K03334] [EC:1.4.3.2]
26275  HIBCH; 3-hydroxyisobutyryl-CoA hydrolase [KO:K05605] [EC:3.1.2.4]
27034  ACAD8; acyl-CoA dehydrogenase family member 8 [KO:K11538] [EC:1.3.99.-]
30  ACAA1; acetyl-CoA acyltransferase 1 [KO:K07513] [EC:2.3.1.16]
3028  HSD17B10; hydroxysteroid 17-beta dehydrogenase 10 [KO:K08683] [EC:1.1.1.35 1.1.1.178]
3030  HADHA; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha [KO:K07515] [EC:4.2.1.17 1.1.1.211]
3032  HADHB; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta [KO:K07509] [EC:2.3.1.16]
3033  HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]
3155  HMGCL; 3-hydroxy-3-methylglutaryl-CoA lyase [KO:K01640] [EC:4.1.3.4]
3157  HMGCS1; 3-hydroxy-3-methylglutaryl-CoA synthase 1 [KO:K01641] [EC:2.3.3.10]
3158  HMGCS2; 3-hydroxy-3-methylglutaryl-CoA synthase 2 [KO:K01641] [EC:2.3.3.10]
316  AOX1; aldehyde oxidase 1 [KO:K00157] [EC:1.2.3.1]
34  ACADM; acyl-CoA dehydrogenase medium chain [KO:K00249] [EC:1.3.8.7]
35  ACADS; acyl-CoA dehydrogenase short chain [KO:K00248] [EC:1.3.8.1]
36  ACADSB; acyl-CoA dehydrogenase short/branched chain [KO:K09478] [EC:1.3.8.5]
3712  IVD; isovaleryl-CoA dehydrogenase [KO:K00253] [EC:1.3.8.4]
38  ACAT1; acetyl-CoA acetyltransferase 1 [KO:K00626] [EC:2.3.1.9]
39  ACAT2; acetyl-CoA acetyltransferase 2 [KO:K00626] [EC:2.3.1.9]
4329  ALDH6A1; aldehyde dehydrogenase 6 family member A1 [KO:K00140] [EC:1.2.1.18 1.2.1.27]
4594  MMUT; methylmalonyl-CoA mutase [KO:K01847] [EC:5.4.99.2]
501  ALDH7A1; aldehyde dehydrogenase 7 family member A1 [KO:K14085] [EC:1.2.1.31 1.2.1.8 1.2.1.3]
5019  OXCT1; 3-oxoacid CoA-transferase 1 [KO:K01027] [EC:2.8.3.5]
5095  PCCA; propionyl-CoA carboxylase subunit alpha [KO:K01965] [EC:6.4.1.3]
5096  PCCB; propionyl-CoA carboxylase subunit beta [KO:K01966] [EC:6.4.1.3 2.1.3.15]
54511  HMGCLL1; 3-hydroxy-3-methylglutaryl-CoA lyase like 1 [KO:K01640] [EC:4.1.3.4]
549  AUH; AU RNA binding methylglutaconyl-CoA hydratase [KO:K05607] [EC:4.2.1.18]
56922  MCCC1; methylcrotonyl-CoA carboxylase subunit 1 [KO:K01968] [EC:6.4.1.4]
586  BCAT1; branched chain amino acid transaminase 1 [KO:K00826] [EC:2.6.1.42]
587  BCAT2; branched chain amino acid transaminase 2 [KO:K00826] [EC:2.6.1.42]
593  BCKDHA; branched chain keto acid dehydrogenase E1 subunit alpha [KO:K00166] [EC:1.2.4.4]
594  BCKDHB; branched chain keto acid dehydrogenase E1 subunit beta [KO:K00167] [EC:1.2.4.4]
64064  OXCT2; 3-oxoacid CoA-transferase 2 [KO:K01027] [EC:2.8.3.5]
64087  MCCC2; methylcrotonyl-CoA carboxylase subunit 2 [KO:K01969] [EC:6.4.1.4]
64902  AGXT2; alanine--glyoxylate aminotransferase 2 [KO:K00827] [EC:2.6.1.44 2.6.1.40]
65985  AACS; acetoacetyl-CoA synthetase [KO:K01907] [EC:6.2.1.16]
84693  MCEE; methylmalonyl-CoA epimerase [KO:K05606] [EC:5.1.99.1]
Compound
C00024  Acetyl-CoA
C00068  Thiamin diphosphate
C00091  Succinyl-CoA
C00100  Propanoyl-CoA
C00123  L-Leucine
C00141  3-Methyl-2-oxobutanoic acid
C00164  Acetoacetate
C00183  L-Valine
C00233  4-Methyl-2-oxopentanoate
C00332  Acetoacetyl-CoA
C00349  2-Methyl-3-oxopropanoate
C00356  (S)-3-Hydroxy-3-methylglutaryl-CoA
C00407  L-Isoleucine
C00630  2-Methylpropanoyl-CoA
C00671  (S)-3-Methyl-2-oxopentanoic acid
C00683  (S)-Methylmalonyl-CoA
C01205  (R)-3-Amino-2-methylpropanoate
C01213  (R)-Methylmalonyl-CoA
C02170  Methylmalonate
C02939  3-Methylbutanoyl-CoA
C03069  3-Methylcrotonyl-CoA
C03231  3-Methylglutaconyl-CoA
C03284  L-3-Aminoisobutanoate
C03344  2-Methylacetoacetyl-CoA
C03345  2-Methylbut-2-enoyl-CoA
C03460  2-Methylprop-2-enoyl-CoA
C04405  (2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA
C05996  Branched chain fatty acid
C05998  3-Hydroxyisovaleryl-CoA
C06000  (S)-3-Hydroxyisobutyryl-CoA
C06001  (S)-3-Hydroxyisobutyrate
C06002  (S)-Methylmalonate semialdehyde
C15972  Enzyme N6-(lipoyl)lysine
C15973  Enzyme N6-(dihydrolipoyl)lysine
C15974  3-Methyl-1-hydroxybutyl-ThPP
C15975  [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(3-methylbutanoyl)dihydrolipoyllysine
C15976  2-Methyl-1-hydroxypropyl-ThPP
C15977  [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine
C15978  2-Methyl-1-hydroxybutyl-ThPP
C15979  [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylbutanoyl)dihydrolipoyllysine
C15980  (S)-2-Methylbutanoyl-CoA
C20827  3-Hydroxyisovalerate
Related
pathway
hsa00020  Citrate cycle (TCA cycle)
hsa00240  Pyrimidine metabolism
hsa00290  Valine, leucine and isoleucine biosynthesis
hsa00640  Propanoate metabolism
hsa00900  Terpenoid backbone biosynthesis
KO pathway
ko00280   

  All links  
No link information was found.
KEGG   PATHWAY: hsa00280
Entry
hsa00280                    Pathway                                
Name
Valine, leucine and isoleucine degradation - Homo sapiens (human)
Class
Metabolism; Amino acid metabolism
Pathway map
hsa00280  Valine, leucine and isoleucine degradation
hsa00280

Module
hsa_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA [PATH:hsa00280]
hsa_M00741  Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA [PATH:hsa00280]
Network
nt06024  Valine, leucine and isoleucine degradation
nt06032  Lipoic acid metabolism
  Element
N00832  Branched-chain amino acids degradation 1
N00842  Propanoyl-CoA metabolism
N00851  Leucine degradation
N00852  Valine degradation
N00856  Isoleucine degradation
N01619  Branched-chain amino acids degradation 2
Other DBs
GO: 0006550 0006552 0006574
Organism
Homo sapiens (human) [GN:hsa]
Gene
10449  ACAA2; acetyl-CoA acyltransferase 2 [KO:K07508] [EC:2.3.1.16]
11112  HIBADH; 3-hydroxyisobutyrate dehydrogenase [KO:K00020] [EC:1.1.1.31]
1629  DBT; dihydrolipoamide branched chain transacylase E2 [KO:K09699] [EC:2.3.1.168]
1738  DLD; dihydrolipoamide dehydrogenase [KO:K00382] [EC:1.8.1.4]
18  ABAT; 4-aminobutyrate aminotransferase [KO:K13524] [EC:2.6.1.19 2.6.1.22]
1892  ECHS1; enoyl-CoA hydratase, short chain 1 [KO:K07511] [EC:4.2.1.17]
1962  EHHADH; enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
197322  ACSF3; acyl-CoA synthetase family member 3 [KO:K18660] [EC:6.2.1.76 6.2.1.-]
217  ALDH2; aldehyde dehydrogenase 2 family member [KO:K00128] [EC:1.2.1.3]
219  ALDH1B1; aldehyde dehydrogenase 1 family member B1 [KO:K00128] [EC:1.2.1.3]
223  ALDH9A1; aldehyde dehydrogenase 9 family member A1 [KO:K00149] [EC:1.2.1.47 1.2.1.3]
224  ALDH3A2; aldehyde dehydrogenase 3 family member A2 [KO:K00128] [EC:1.2.1.3]
259307  IL4I1; interleukin 4 induced 1 [KO:K03334] [EC:1.4.3.2]
26275  HIBCH; 3-hydroxyisobutyryl-CoA hydrolase [KO:K05605] [EC:3.1.2.4]
27034  ACAD8; acyl-CoA dehydrogenase family member 8 [KO:K11538] [EC:1.3.99.-]
30  ACAA1; acetyl-CoA acyltransferase 1 [KO:K07513] [EC:2.3.1.16]
3028  HSD17B10; hydroxysteroid 17-beta dehydrogenase 10 [KO:K08683] [EC:1.1.1.35 1.1.1.178]
3030  HADHA; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha [KO:K07515] [EC:4.2.1.17 1.1.1.211]
3032  HADHB; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta [KO:K07509] [EC:2.3.1.16]
3033  HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]
3155  HMGCL; 3-hydroxy-3-methylglutaryl-CoA lyase [KO:K01640] [EC:4.1.3.4]
3157  HMGCS1; 3-hydroxy-3-methylglutaryl-CoA synthase 1 [KO:K01641] [EC:2.3.3.10]
3158  HMGCS2; 3-hydroxy-3-methylglutaryl-CoA synthase 2 [KO:K01641] [EC:2.3.3.10]
316  AOX1; aldehyde oxidase 1 [KO:K00157] [EC:1.2.3.1]
34  ACADM; acyl-CoA dehydrogenase medium chain [KO:K00249] [EC:1.3.8.7]
35  ACADS; acyl-CoA dehydrogenase short chain [KO:K00248] [EC:1.3.8.1]
36  ACADSB; acyl-CoA dehydrogenase short/branched chain [KO:K09478] [EC:1.3.8.5]
3712  IVD; isovaleryl-CoA dehydrogenase [KO:K00253] [EC:1.3.8.4]
38  ACAT1; acetyl-CoA acetyltransferase 1 [KO:K00626] [EC:2.3.1.9]
39  ACAT2; acetyl-CoA acetyltransferase 2 [KO:K00626] [EC:2.3.1.9]
4329  ALDH6A1; aldehyde dehydrogenase 6 family member A1 [KO:K00140] [EC:1.2.1.18 1.2.1.27]
4594  MMUT; methylmalonyl-CoA mutase [KO:K01847] [EC:5.4.99.2]
501  ALDH7A1; aldehyde dehydrogenase 7 family member A1 [KO:K14085] [EC:1.2.1.31 1.2.1.8 1.2.1.3]
5019  OXCT1; 3-oxoacid CoA-transferase 1 [KO:K01027] [EC:2.8.3.5]
5095  PCCA; propionyl-CoA carboxylase subunit alpha [KO:K01965] [EC:6.4.1.3]
5096  PCCB; propionyl-CoA carboxylase subunit beta [KO:K01966] [EC:6.4.1.3 2.1.3.15]
54511  HMGCLL1; 3-hydroxy-3-methylglutaryl-CoA lyase like 1 [KO:K01640] [EC:4.1.3.4]
549  AUH; AU RNA binding methylglutaconyl-CoA hydratase [KO:K05607] [EC:4.2.1.18]
56922  MCCC1; methylcrotonyl-CoA carboxylase subunit 1 [KO:K01968] [EC:6.4.1.4]
586  BCAT1; branched chain amino acid transaminase 1 [KO:K00826] [EC:2.6.1.42]
587  BCAT2; branched chain amino acid transaminase 2 [KO:K00826] [EC:2.6.1.42]
593  BCKDHA; branched chain keto acid dehydrogenase E1 subunit alpha [KO:K00166] [EC:1.2.4.4]
594  BCKDHB; branched chain keto acid dehydrogenase E1 subunit beta [KO:K00167] [EC:1.2.4.4]
64064  OXCT2; 3-oxoacid CoA-transferase 2 [KO:K01027] [EC:2.8.3.5]
64087  MCCC2; methylcrotonyl-CoA carboxylase subunit 2 [KO:K01969] [EC:6.4.1.4]
64902  AGXT2; alanine--glyoxylate aminotransferase 2 [KO:K00827] [EC:2.6.1.44 2.6.1.40]
65985  AACS; acetoacetyl-CoA synthetase [KO:K01907] [EC:6.2.1.16]
84693  MCEE; methylmalonyl-CoA epimerase [KO:K05606] [EC:5.1.99.1]
Compound
C00024  Acetyl-CoA
C00068  Thiamin diphosphate
C00091  Succinyl-CoA
C00100  Propanoyl-CoA
C00123  L-Leucine
C00141  3-Methyl-2-oxobutanoic acid
C00164  Acetoacetate
C00183  L-Valine
C00233  4-Methyl-2-oxopentanoate
C00332  Acetoacetyl-CoA
C00349  2-Methyl-3-oxopropanoate
C00356  (S)-3-Hydroxy-3-methylglutaryl-CoA
C00407  L-Isoleucine
C00630  2-Methylpropanoyl-CoA
C00671  (S)-3-Methyl-2-oxopentanoic acid
C00683  (S)-Methylmalonyl-CoA
C01205  (R)-3-Amino-2-methylpropanoate
C01213  (R)-Methylmalonyl-CoA
C02170  Methylmalonate
C02939  3-Methylbutanoyl-CoA
C03069  3-Methylcrotonyl-CoA
C03231  3-Methylglutaconyl-CoA
C03284  L-3-Aminoisobutanoate
C03344  2-Methylacetoacetyl-CoA
C03345  2-Methylbut-2-enoyl-CoA
C03460  2-Methylprop-2-enoyl-CoA
C04405  (2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA
C05996  Branched chain fatty acid
C05998  3-Hydroxyisovaleryl-CoA
C06000  (S)-3-Hydroxyisobutyryl-CoA
C06001  (S)-3-Hydroxyisobutyrate
C06002  (S)-Methylmalonate semialdehyde
C15972  Enzyme N6-(lipoyl)lysine
C15973  Enzyme N6-(dihydrolipoyl)lysine
C15974  3-Methyl-1-hydroxybutyl-ThPP
C15975  [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(3-methylbutanoyl)dihydrolipoyllysine
C15976  2-Methyl-1-hydroxypropyl-ThPP
C15977  [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine
C15978  2-Methyl-1-hydroxybutyl-ThPP
C15979  [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylbutanoyl)dihydrolipoyllysine
C15980  (S)-2-Methylbutanoyl-CoA
C20827  3-Hydroxyisovalerate
Related
pathway
hsa00020  Citrate cycle (TCA cycle)
hsa00240  Pyrimidine metabolism
hsa00290  Valine, leucine and isoleucine biosynthesis
hsa00640  Propanoate metabolism
hsa00900  Terpenoid backbone biosynthesis
KO pathway
ko00280   

  All links  
No link information was found.
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