KEGG   PATHWAY: uah00280
Entry
uah00280                    Pathway                                
Name
Valine, leucine and isoleucine degradation - Ursus arctos (brown bear)
Class
Metabolism; Amino acid metabolism
Pathway map
uah00280  Valine, leucine and isoleucine degradation
uah00280

Module
uah_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA [PATH:uah00280]
uah_M00741  Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA [PATH:uah00280]
Other DBs
GO: 0006550 0006552 0006574
Organism
Ursus arctos (brown bear) [GN:uah]
Gene
113243329  BCAT2; branched-chain-amino-acid aminotransferase, mitochondrial isoform X1 [KO:K00826] [EC:2.6.1.42]
113257794  BCAT1; branched-chain-amino-acid aminotransferase, cytosolic isoform X1 [KO:K00826] [EC:2.6.1.42]
113254224  L-amino-acid oxidase-like [KO:K03334] [EC:1.4.3.2]
113243274  IL4I1; L-amino-acid oxidase isoform X1 [KO:K03334] [EC:1.4.3.2]
113243532  BCKDHA; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform X1 [KO:K00166] [EC:1.2.4.4]
113261078  BCKDHB; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X1 [KO:K00167] [EC:1.2.4.4]
113254355  DBT; lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial isoform X1 [KO:K09699] [EC:2.3.1.168]
113258483  DLD; dihydrolipoyl dehydrogenase, mitochondrial [KO:K00382] [EC:1.8.1.4]
113267053  ACADS; short-chain specific acyl-CoA dehydrogenase, mitochondrial [KO:K00248] [EC:1.3.8.1]
113254520  ACADM; medium-chain specific acyl-CoA dehydrogenase, mitochondrial [KO:K00249] [EC:1.3.8.7]
113242033  IVD; isovaleryl-CoA dehydrogenase, mitochondrial isoform X1 [KO:K00253] [EC:1.3.8.4]
113252021  ACADSB; short/branched chain specific acyl-CoA dehydrogenase, mitochondrial isoform X1 [KO:K09478] [EC:1.3.8.5]
113259847  ACAD8; isobutyryl-CoA dehydrogenase, mitochondrial [KO:K11538] [EC:1.3.99.-]
113270772  HADHA; trifunctional enzyme subunit alpha, mitochondrial isoform X1 [KO:K07515] [EC:4.2.1.17 1.1.1.211]
113254101  EHHADH; peroxisomal bifunctional enzyme isoform X1 [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
113252096  ECHS1; enoyl-CoA hydratase, mitochondrial [KO:K07511] [EC:4.2.1.17]
113255543  HADH; hydroxyacyl-coenzyme A dehydrogenase, mitochondrial [KO:K00022] [EC:1.1.1.35]
113247518  HSD17B10; 3-hydroxyacyl-CoA dehydrogenase type-2 [KO:K08683] [EC:1.1.1.35 1.1.1.178]
113253780  ACAA1; 3-ketoacyl-CoA thiolase, peroxisomal [KO:K07513] [EC:2.3.1.16]
113253408  ACAA2; 3-ketoacyl-CoA thiolase, mitochondrial isoform X6 [KO:K07508] [EC:2.3.1.16]
113270781  HADHB; trifunctional enzyme subunit beta, mitochondrial isoform X5 [KO:K07509] [EC:2.3.1.16]
113251504  PCCA; propionyl-CoA carboxylase alpha chain, mitochondrial isoform X1 [KO:K01965] [EC:6.4.1.3]
113253970  PCCB; propionyl-CoA carboxylase beta chain, mitochondrial isoform X2 [KO:K01966] [EC:6.4.1.3 2.1.3.15]
113263613  MCEE; methylmalonyl-CoA epimerase, mitochondrial [KO:K05606] [EC:5.1.99.1]
113261210  MMUT; methylmalonyl-CoA mutase, mitochondrial isoform X2 [KO:K01847] [EC:5.4.99.2]
113250659  HIBCH; 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial [KO:K05605] [EC:3.1.2.4]
113262129  HIBADH; 3-hydroxyisobutyrate dehydrogenase, mitochondrial [KO:K00020] [EC:1.1.1.31]
113270345  ALDH6A1; methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial [KO:K00140] [EC:1.2.1.18 1.2.1.27]
113271270  ALDH3A2; aldehyde dehydrogenase family 3 member A2 isoform X3 [KO:K00128] [EC:1.2.1.3]
113260433  ALDH1B1; LOW QUALITY PROTEIN: aldehyde dehydrogenase X, mitochondrial [KO:K00128] [EC:1.2.1.3]
113266971  ALDH2; aldehyde dehydrogenase, mitochondrial isoform X2 [KO:K00128] [EC:1.2.1.3]
113243301  ALDH16A1; aldehyde dehydrogenase family 16 member A1 isoform X1 [KO:K00128] [EC:1.2.1.3]
113261663  ALDH7A1; alpha-aminoadipic semialdehyde dehydrogenase [KO:K14085] [EC:1.2.1.31 1.2.1.8 1.2.1.3]
113268692  ALDH9A1; 4-trimethylaminobutyraldehyde dehydrogenase isoform X1 [KO:K00149] [EC:1.2.1.47 1.2.1.3]
113250605  aldehyde oxidase 2 isoform X2 [KO:K00157] [EC:1.2.3.1]
113250606  AOX1; aldehyde oxidase isoform X2 [KO:K00157] [EC:1.2.3.1]
113250607  aldehyde oxidase 4-like [KO:K00157] [EC:1.2.3.1]
113252139  ACSF3; malonate--CoA ligase ACSF3, mitochondrial isoform X1 [KO:K18660] [EC:6.2.1.76 6.2.1.-]
113270819  ABAT; 4-aminobutyrate aminotransferase, mitochondrial [KO:K13524] [EC:2.6.1.19 2.6.1.22]
113260880  AGXT2; alanine--glyoxylate aminotransferase 2, mitochondrial isoform X1 [KO:K00827] [EC:2.6.1.44 2.6.1.40]
113254564  MCCC1; methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial [KO:K01968] [EC:6.4.1.4]
113255242  MCCC2; methylcrotonoyl-CoA carboxylase beta chain, mitochondrial isoform X1 [KO:K01969] [EC:6.4.1.4]
113270135  AUH; methylglutaconyl-CoA hydratase, mitochondrial isoform X1 [KO:K05607] [EC:4.2.1.18]
113268515  HMGCL; hydroxymethylglutaryl-CoA lyase, mitochondrial [KO:K01640] [EC:4.1.3.4]
113261167  HMGCLL1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic isoform X1 [KO:K01640] [EC:4.1.3.4]
113260937  OXCT1; succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial isoform X1 [KO:K01027] [EC:2.8.3.5]
125283081  OXCT2; LOW QUALITY PROTEIN: succinyl-CoA:3-ketoacid coenzyme A transferase 2, mitochondrial [KO:K01027] [EC:2.8.3.5]
113266870  AACS; acetoacetyl-CoA synthetase isoform X1 [KO:K01907] [EC:6.2.1.16]
113260100  ACAT1; acetyl-CoA acetyltransferase, mitochondrial [KO:K00626] [EC:2.3.1.9]
113259619  ACAT2; acetyl-CoA acetyltransferase, cytosolic [KO:K00626] [EC:2.3.1.9]
113244359  HMGCS2; hydroxymethylglutaryl-CoA synthase, mitochondrial [KO:K01641] [EC:2.3.3.10]
113260955  HMGCS1; hydroxymethylglutaryl-CoA synthase, cytoplasmic [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
Related
pathway
uah00020  Citrate cycle (TCA cycle)
uah00240  Pyrimidine metabolism
uah00290  Valine, leucine and isoleucine biosynthesis
uah00640  Propanoate metabolism
uah00900  Terpenoid backbone biosynthesis
KO pathway
ko00280   
LinkDB

DBGET integrated database retrieval system