KEGG   PATHWAY: oas00280
Entry
oas00280                    Pathway                                
Name
Valine, leucine and isoleucine degradation - Ovis aries (sheep)
Class
Metabolism; Amino acid metabolism
Pathway map
oas00280  Valine, leucine and isoleucine degradation
oas00280

Module
oas_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA [PATH:oas00280]
oas_M00741  Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA [PATH:oas00280]
Other DBs
GO: 0006550 0006552 0006574
Organism
Ovis aries (sheep) [GN:oas]
Gene
100145857  BCKDHA; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial [KO:K00166] [EC:1.2.4.4]
100192316  HADHA; trifunctional enzyme subunit alpha, mitochondrial [KO:K07515] [EC:4.2.1.17 1.1.1.211]
101102177  OXCT1; succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial isoform X2 [KO:K01027] [EC:2.8.3.5]
101102548  [KO:K00157] [EC:1.2.3.1]
101102606  ACADSB; short/branched chain specific acyl-CoA dehydrogenase, mitochondrial isoform X1 [KO:K09478] [EC:1.3.8.5]
101103614  [KO:K03334] [EC:1.4.3.2]
101104557  [KO:K00157] [EC:1.2.3.1]
101104601  ACAT1; acetyl-CoA acetyltransferase, mitochondrial [KO:K00626] [EC:2.3.1.9]
101104808  [KO:K00157] [EC:1.2.3.1]
101105060  ALDH1B1; LOW QUALITY PROTEIN: aldehyde dehydrogenase X, mitochondrial [KO:K00128] [EC:1.2.1.3]
101105152  DLD; dihydrolipoyl dehydrogenase, mitochondrial isoform X2 [KO:K00382] [EC:1.8.1.4]
101105462  ABAT; 4-aminobutyrate aminotransferase, mitochondrial [KO:K13524] [EC:2.6.1.19 2.6.1.22]
101106176  ALDH6A1; methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial [KO:K00140] [EC:1.2.1.18 1.2.1.27]
101108127  ALDH7A1; alpha-aminoadipic semialdehyde dehydrogenase isoform X2 [KO:K14085] [EC:1.2.1.31 1.2.1.8 1.2.1.3]
101109383  ACSF3; malonate--CoA ligase ACSF3, mitochondrial isoform X1 [KO:K18660] [EC:6.2.1.76 6.2.1.-]
101109411  ALDH9A1; 4-trimethylaminobutyraldehyde dehydrogenase isoform X1 [KO:K00149] [EC:1.2.1.47 1.2.1.3]
101109520  AUH; methylglutaconyl-CoA hydratase, mitochondrial isoform X1 [KO:K05607] [EC:4.2.1.18]
101109578  [KO:K00020] [EC:1.1.1.31]
101109685  HADHB; trifunctional enzyme subunit beta, mitochondrial isoform X2 [KO:K07509] [EC:2.3.1.16]
101110023  MCCC1; methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform X4 [KO:K01968] [EC:6.4.1.4]
101110625  ACAA1; 3-ketoacyl-CoA thiolase, peroxisomal [KO:K07513] [EC:2.3.1.16]
101111161  HIBCH; 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial isoform X1 [KO:K05605] [EC:3.1.2.4]
101111590  HMGCS2; hydroxymethylglutaryl-CoA synthase, mitochondrial [KO:K01641] [EC:2.3.3.10]
101111660  AGXT2; alanine--glyoxylate aminotransferase 2, mitochondrial isoform X1 [KO:K00827] [EC:2.6.1.44 2.6.1.40]
101111900  ALDH16A1; aldehyde dehydrogenase family 16 member A1 [KO:K00128] [EC:1.2.1.3]
101111925  ACAA2; 3-ketoacyl-CoA thiolase, mitochondrial isoform X6 [KO:K07508] [EC:2.3.1.16]
101111975  ACAT2; acetyl-CoA acetyltransferase, cytosolic [KO:K00626] [EC:2.3.1.9]
101112867  ECHS1; enoyl-CoA hydratase, mitochondrial isoform X2 [KO:K07511] [EC:4.2.1.17]
101112991  PCCA; propionyl-CoA carboxylase alpha chain, mitochondrial isoform X2 [KO:K01965] [EC:6.4.1.3]
101113492  HIBADH; 3-hydroxyisobutyrate dehydrogenase, mitochondrial [KO:K00020] [EC:1.1.1.31]
101113541  HMGCLL1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic [KO:K01640] [EC:4.1.3.4]
101113555  PCCB; propionyl-CoA carboxylase beta chain, mitochondrial isoform X2 [KO:K01966] [EC:6.4.1.3 2.1.3.15]
101113789  ACAD8; isobutyryl-CoA dehydrogenase, mitochondrial [KO:K11538] [EC:1.3.99.-]
101113856  IVD; isovaleryl-CoA dehydrogenase, mitochondrial [KO:K00253] [EC:1.3.8.4]
101113939  BCKDHB; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial [KO:K00167] [EC:1.2.4.4]
101114977  AACS; acetoacetyl-CoA synthetase isoform X4 [KO:K01907] [EC:6.2.1.16]
101116511  ALDH2; aldehyde dehydrogenase, mitochondrial isoform X2 [KO:K00128] [EC:1.2.1.3]
101116765  MCCC2; methylcrotonoyl-CoA carboxylase beta chain, mitochondrial [KO:K01969] [EC:6.4.1.4]
101117635  EHHADH; peroxisomal bifunctional enzyme [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
101117702  MCEE; methylmalonyl-CoA epimerase, mitochondrial [KO:K05606] [EC:5.1.99.1]
101118798  ALDH3A2; aldehyde dehydrogenase family 3 member A2 isoform X1 [KO:K00128] [EC:1.2.1.3]
101119699  HMGCL; hydroxymethylglutaryl-CoA lyase, mitochondrial [KO:K01640] [EC:4.1.3.4]
101119850  ACADS; short-chain specific acyl-CoA dehydrogenase, mitochondrial [KO:K00248] [EC:1.3.8.1]
101119880  HSD17B10; 3-hydroxyacyl-CoA dehydrogenase type-2 [KO:K08683] [EC:1.1.1.35 1.1.1.178]
101120981  DBT; lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial isoform X1 [KO:K09699] [EC:2.3.1.168]
101122731  MMUT; methylmalonyl-CoA mutase, mitochondrial [KO:K01847] [EC:5.4.99.2]
101122862  HADH; hydroxyacyl-coenzyme A dehydrogenase, mitochondrial isoform X2 [KO:K00022] [EC:1.1.1.35]
101122979  HMGCS1; hydroxymethylglutaryl-CoA synthase, cytoplasmic isoform X2 [KO:K01641] [EC:2.3.3.10]
105602079  IL4I1; L-amino-acid oxidase isoform X1 [KO:K03334] [EC:1.4.3.2]
105602405  [KO:K09699] [EC:2.3.1.168]
105604051  [KO:K01027] [EC:2.8.3.5]
105605147  OXCT2; succinyl-CoA:3-ketoacid coenzyme A transferase 2, mitochondrial [KO:K01027] [EC:2.8.3.5]
114116805  ACADM; medium-chain specific acyl-CoA dehydrogenase, mitochondrial isoform X1 [KO:K00249] [EC:1.3.8.7]
443450  BCAT2; branched-chain-amino-acid aminotransferase, mitochondrial isoform X2 [KO:K00826] [EC:2.6.1.42]
443485  BCAT1; branched-chain-amino-acid aminotransferase, cytosolic [KO:K00826] [EC:2.6.1.42]
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
oas00020  Citrate cycle (TCA cycle)
oas00240  Pyrimidine metabolism
oas00290  Valine, leucine and isoleucine biosynthesis
oas00640  Propanoate metabolism
oas00900  Terpenoid backbone biosynthesis
KO pathway
ko00280   

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