KEGG   PATHWAY: hsa00280Help
Entry
hsa00280                    Pathway                                

Name
Valine, leucine and isoleucine degradation - Homo sapiens (human)
Class
Metabolism; Amino acid metabolism
BRITE hierarchy
Pathway map
hsa00280  Valine, leucine and isoleucine degradation
hsa00280

Ortholog table
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
  Element
N00832  Branched-chain amino acids degradation
N00833  HMGCL deficiency in leucine degradation
N00834  AUH deficiency in leucine degradation
N00835  MCCC1 deficiency in leucine degradation
N00836  MCCC2 deficiency in leucine degradation
N00837  IVD deficiency in leucine degradation
N00838  BCKDHA deficiency in branched-chain amino acids degradation
N00839  BCKDHB deficiency in branched-chain amino acids degradation
N00840  DBT deficiency in branched-chain amino acids degradation
N00841  DLD deficiency in branched-chain amino acids degradation
N00842  Propanoyl-CoA metabolism
N00843  MMUT deficiency in propanoyl-CoA metabolism
N00844  MCEE deficiency in propanoyl-CoA metabolism
N00845  PCCA deficiency in propanoyl-CoA metabolism
N00846  PCCB deficiency in propanoyl-CoA metabolism
N00851  Leucine degradation
N00852  Valine degradation
N00853  ALDH6A1 deficiency in valine degradation
N00854  HIBCH deficiency in valine degradation
N00855  ACAD8 deficiency in valine degradation
N00856  Isoleucine degradation
N00857  HSD17B10 deficiency in isoleucine degradation
N00858  ACADSB deficiency in isoleucine degradation
Disease
H00172  Maple syrup urine disease
H00173  Isovaleric acidemia
H00174  Methylmalonic aciduria
H00175  Propionic acidemia
H00179  3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
H00181  3-Methylcrotonylglycinuria
H00375  SBCAD deficiency
H00754  3-Methylglutaconic aciduria
H00925  2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency
H01076  Alpha-methylacetoacetic aciduria
H01121  Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency
H01123  HMG-CoA synthase deficiency
H01279  Isobutyryl-CoA dehydrogenase deficiency
H01349  Methacrylic aciduria
H01364  3-Hydroxyacyl-CoA dehydrogenase deficiency
H01400  Secondary hyperammonemia
H02000  Dihydrolipoamide dehydrogenase deficiency
H02109  Combined malonic and methylmalonic aciduria
H02285  Methylmalonate semialdehyde dehydrogenase deficiency
Other DBs
GO: 0006550 0006552 0006574
Organism
Homo sapiens (human) [GN:hsa]
Gene
587  BCAT2; branched chain amino acid transaminase 2 [KO:K00826] [EC:2.6.1.42]
586  BCAT1; branched chain amino acid transaminase 1 [KO:K00826] [EC:2.6.1.42]
259307  IL4I1; interleukin 4 induced 1 [KO:K03334] [EC:1.4.3.2]
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]
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]
35  ACADS; acyl-CoA dehydrogenase short chain [KO:K00248] [EC:1.3.8.1]
34  ACADM; acyl-CoA dehydrogenase medium chain [KO:K00249] [EC:1.3.8.7]
3712  IVD; isovaleryl-CoA dehydrogenase [KO:K00253] [EC:1.3.8.4]
36  ACADSB; acyl-CoA dehydrogenase short/branched chain [KO:K09478] [EC:1.3.99.12]
27034  ACAD8; acyl-CoA dehydrogenase family member 8 [KO:K11538] [EC:1.3.99.-]
3030  HADHA; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha [KO:K07515] [EC:4.2.1.17 1.1.1.211]
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]
1892  ECHS1; enoyl-CoA hydratase, short chain 1 [KO:K07511] [EC:4.2.1.17]
3033  HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]
3028  HSD17B10; hydroxysteroid 17-beta dehydrogenase 10 [KO:K08683] [EC:1.1.1.35 1.1.1.178]
30  ACAA1; acetyl-CoA acyltransferase 1 [KO:K07513] [EC:2.3.1.16]
10449  ACAA2; acetyl-CoA acyltransferase 2 [KO:K07508] [EC:2.3.1.16]
3032  HADHB; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta [KO:K07509] [EC:2.3.1.16]
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]
84693  MCEE; methylmalonyl-CoA epimerase [KO:K05606] [EC:5.1.99.1]
4594  MMUT; methylmalonyl-CoA mutase [KO:K01847] [EC:5.4.99.2]
26275  HIBCH; 3-hydroxyisobutyryl-CoA hydrolase [KO:K05605] [EC:3.1.2.4]
11112  HIBADH; 3-hydroxyisobutyrate dehydrogenase [KO:K00020] [EC:1.1.1.31]
4329  ALDH6A1; aldehyde dehydrogenase 6 family member A1 [KO:K00140] [EC:1.2.1.18 1.2.1.27]
217  ALDH2; aldehyde dehydrogenase 2 family member [KO:K00128] [EC:1.2.1.3]
224  ALDH3A2; aldehyde dehydrogenase 3 family member A2 [KO:K00128] [EC:1.2.1.3]
219  ALDH1B1; aldehyde dehydrogenase 1 family member B1 [KO:K00128] [EC:1.2.1.3]
501  ALDH7A1; aldehyde dehydrogenase 7 family member A1 [KO:K14085] [EC:1.2.1.31 1.2.1.8 1.2.1.3]
223  ALDH9A1; aldehyde dehydrogenase 9 family member A1 [KO:K00149] [EC:1.2.1.47 1.2.1.3]
316  AOX1; aldehyde oxidase 1 [KO:K00157] [EC:1.2.3.1]
197322  ACSF3; acyl-CoA synthetase family member 3 [KO:K18660] [EC:6.2.1.-]
18  ABAT; 4-aminobutyrate aminotransferase [KO:K13524] [EC:2.6.1.19 2.6.1.22]
64902  AGXT2; alanine--glyoxylate aminotransferase 2 [KO:K00827] [EC:2.6.1.44 2.6.1.40]
56922  MCCC1; methylcrotonoyl-CoA carboxylase 1 [KO:K01968] [EC:6.4.1.4]
64087  MCCC2; methylcrotonoyl-CoA carboxylase 2 [KO:K01969] [EC:6.4.1.4]
549  AUH; AU RNA binding methylglutaconyl-CoA hydratase [KO:K05607] [EC:4.2.1.18]
3155  HMGCL; 3-hydroxy-3-methylglutaryl-CoA lyase [KO:K01640] [EC:4.1.3.4]
54511  HMGCLL1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase like 1 [KO:K01640] [EC:4.1.3.4]
5019  OXCT1; 3-oxoacid CoA-transferase 1 [KO:K01027] [EC:2.8.3.5]
64064  OXCT2; 3-oxoacid CoA-transferase 2 [KO:K01027] [EC:2.8.3.5]
65985  AACS; acetoacetyl-CoA synthetase [KO:K01907] [EC:6.2.1.16]
39  ACAT2; acetyl-CoA acetyltransferase 2 [KO:K00626] [EC:2.3.1.9]
38  ACAT1; acetyl-CoA acetyltransferase 1 [KO:K00626] [EC:2.3.1.9]
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]
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
KO pathway
ko00280   

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