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; 3-ketoacyl-CoA thiolase, mitochondrial [KO:K07508] [EC:2.3.1.16]
11112  HIBADH; 3-hydroxyisobutyrate dehydrogenase, mitochondrial isoform 1 precursor [KO:K00020] [EC:1.1.1.31]
1629  DBT; lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial isoform 1 precursor [KO:K09699] [EC:2.3.1.168]
1738  DLD; dihydrolipoyl dehydrogenase, mitochondrial isoform 1 precursor [KO:K00382] [EC:1.8.1.4]
18  ABAT; 4-aminobutyrate aminotransferase, mitochondrial isoform 1 precursor [KO:K13524] [EC:2.6.1.19 2.6.1.22]
1892  ECHS1; enoyl-CoA hydratase, mitochondrial [KO:K07511] [EC:4.2.1.17]
1962  EHHADH; peroxisomal bifunctional enzyme isoform 1 [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
197322  ACSF3; malonate--CoA ligase ACSF3, mitochondrial isoform 1 precursor [KO:K18660] [EC:6.2.1.76 6.2.1.-]
217  ALDH2; aldehyde dehydrogenase, mitochondrial isoform 1 precursor [KO:K00128] [EC:1.2.1.3]
219  ALDH1B1; aldehyde dehydrogenase X, mitochondrial precursor [KO:K00128] [EC:1.2.1.3]
223  ALDH9A1; 4-trimethylaminobutyraldehyde dehydrogenase isoform 1 precursor [KO:K00149] [EC:1.2.1.47 1.2.1.3]
224  ALDH3A2; aldehyde dehydrogenase family 3 member A2 isoform 2 [KO:K00128] [EC:1.2.1.3]
259307  IL4I1; L-amino-acid oxidase isoform 1 precursor [KO:K03334] [EC:1.4.3.2]
26275  HIBCH; 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial isoform 1 precursor [KO:K05605] [EC:3.1.2.4]
27034  ACAD8; isobutyryl-CoA dehydrogenase, mitochondrial isoform 2 [KO:K11538] [EC:1.3.99.-]
30  ACAA1; 3-ketoacyl-CoA thiolase, peroxisomal isoform a [KO:K07513] [EC:2.3.1.16]
3028  HSD17B10; 3-hydroxyacyl-CoA dehydrogenase type-2 isoform 1 [KO:K08683] [EC:1.1.1.35 1.1.1.178]
3030  HADHA; trifunctional enzyme subunit alpha, mitochondrial precursor [KO:K07515] [EC:4.2.1.17 1.1.1.211]
3032  HADHB; trifunctional enzyme subunit beta, mitochondrial isoform 1 precursor [KO:K07509] [EC:2.3.1.16]
3033  HADH; hydroxyacyl-coenzyme A dehydrogenase, mitochondrial isoform 2 precursor [KO:K00022] [EC:1.1.1.35]
3155  HMGCL; hydroxymethylglutaryl-CoA lyase, mitochondrial isoform 1 precursor [KO:K01640] [EC:4.1.3.4]
3157  HMGCS1; hydroxymethylglutaryl-CoA synthase, cytoplasmic isoform 1 [KO:K01641] [EC:2.3.3.10]
3158  HMGCS2; hydroxymethylglutaryl-CoA synthase, mitochondrial isoform 1 precursor [KO:K01641] [EC:2.3.3.10]
316  AOX1; aldehyde oxidase [KO:K00157] [EC:1.2.3.1]
34  ACADM; medium-chain specific acyl-CoA dehydrogenase, mitochondrial isoform a precursor [KO:K00249] [EC:1.3.8.7]
35  ACADS; short-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1 precursor [KO:K00248] [EC:1.3.8.1]
36  ACADSB; short/branched chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1 precursor [KO:K09478] [EC:1.3.8.5]
3712  IVD; isovaleryl-CoA dehydrogenase, mitochondrial isoform 1 precursor [KO:K00253] [EC:1.3.8.4]
38  ACAT1; acetyl-CoA acetyltransferase, mitochondrial isoform b precursor [KO:K00626] [EC:2.3.1.9]
39  ACAT2; acetyl-CoA acetyltransferase, cytosolic isoform 1 [KO:K00626] [EC:2.3.1.9]
4329  ALDH6A1; methylmalonate-semialdehyde/malonate-semialdehyde dehydrogenase [acylating], mitochondrial isoform 1 precursor [KO:K00140] [EC:1.2.1.18 1.2.1.27]
4594  MMUT; methylmalonyl-CoA mutase, mitochondrial precursor [KO:K01847] [EC:5.4.99.2]
501  ALDH7A1; alpha-aminoadipic semialdehyde dehydrogenase isoform 1 precursor [KO:K14085] [EC:1.2.1.31 1.2.1.8 1.2.1.3]
5019  OXCT1; succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial isoform 3 precursor [KO:K01027] [EC:2.8.3.5]
5095  PCCA; propionyl-CoA carboxylase alpha chain, mitochondrial isoform a precursor [KO:K01965] [EC:6.4.1.3]
5096  PCCB; propionyl-CoA carboxylase beta chain, mitochondrial isoform 1 precursor [KO:K01966] [EC:6.4.1.3 2.1.3.15]
54511  HMGCLL1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic isoform a [KO:K01640] [EC:4.1.3.4]
549  AUH; methylglutaconyl-CoA hydratase, mitochondrial isoform 1 precursor [KO:K05607] [EC:4.2.1.18]
56922  MCCC1; methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 precursor [KO:K01968] [EC:6.4.1.4]
586  BCAT1; branched-chain-amino-acid aminotransferase, cytosolic isoform 1 [KO:K00826] [EC:2.6.1.42]
587  BCAT2; branched-chain-amino-acid aminotransferase, mitochondrial isoform a precursor [KO:K00826] [EC:2.6.1.42]
593  BCKDHA; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1 precursor [KO:K00166] [EC:1.2.4.4]
594  BCKDHB; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform 1 precursor [KO:K00167] [EC:1.2.4.4]
64064  OXCT2; succinyl-CoA:3-ketoacid coenzyme A transferase 2, mitochondrial precursor [KO:K01027] [EC:2.8.3.5]
64087  MCCC2; methylcrotonoyl-CoA carboxylase beta chain, mitochondrial isoform 1 precursor [KO:K01969] [EC:6.4.1.4]
64902  AGXT2; alanine--glyoxylate aminotransferase 2, mitochondrial isoform a precursor [KO:K00827] [EC:2.6.1.44 2.6.1.40]
65985  AACS; acetoacetyl-CoA synthetase isoform 1 [KO:K01907] [EC:6.2.1.16]
84693  MCEE; methylmalonyl-CoA epimerase, mitochondrial precursor [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   

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