KEGG   PATHWAY: mmma00280
Entry
mmma00280                   Pathway                                
Name
Valine, leucine and isoleucine degradation - Marmota marmota marmota (Alpine marmot)
Class
Metabolism; Amino acid metabolism
Pathway map
mmma00280  Valine, leucine and isoleucine degradation
mmma00280

Module
mmma_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA [PATH:mmma00280]
mmma_M00741  Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA [PATH:mmma00280]
Other DBs
GO: 0006550 0006552 0006574
Organism
Marmota marmota marmota (Alpine marmot) [GN:mmma]
Gene
107137442  Hadha; trifunctional enzyme subunit alpha, mitochondrial [KO:K07515] [EC:4.2.1.17 1.1.1.211]
107137443  Hadhb; trifunctional enzyme subunit beta, mitochondrial [KO:K07509] [EC:2.3.1.16]
107138228  Ivd; isovaleryl-CoA dehydrogenase, mitochondrial [KO:K00253] [EC:1.3.8.4]
107138348  Hibch; 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial [KO:K05605] [EC:3.1.2.4]
107138360  [KO:K00157] [EC:1.2.3.1]
107138590  Aox1; aldehyde oxidase isoform X1 [KO:K00157] [EC:1.2.3.1]
107138591  [KO:K00157] [EC:1.2.3.1]
107139510  Acaa2; 3-ketoacyl-CoA thiolase, mitochondrial isoform X1 [KO:K07508] [EC:2.3.1.16]
107139793  [KO:K01027] [EC:2.8.3.5]
107140928  Pcca; propionyl-CoA carboxylase alpha chain, mitochondrial isoform X1 [KO:K01965] [EC:6.4.1.3]
107141376  Hadh; hydroxyacyl-coenzyme A dehydrogenase, mitochondrial isoform X1 [KO:K00022] [EC:1.1.1.35]
107141477  [KO:K03334] [EC:1.4.3.2]
107141491  Oxct2; LOW QUALITY PROTEIN: succinyl-CoA:3-ketoacid coenzyme A transferase 2, mitochondrial [KO:K01027] [EC:2.8.3.5]
107142132  Hmgcl; hydroxymethylglutaryl-CoA lyase, mitochondrial [KO:K01640] [EC:4.1.3.4]
107143029  Aldh7a1; alpha-aminoadipic semialdehyde dehydrogenase [KO:K14085] [EC:1.2.1.31 1.2.1.8 1.2.1.3]
107143372  Pccb; propionyl-CoA carboxylase beta chain, mitochondrial [KO:K01966] [EC:6.4.1.3 2.1.3.15]
107143678  Hmgcll1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic [KO:K01640] [EC:4.1.3.4]
107143983  Mmut; methylmalonyl-CoA mutase, mitochondrial isoform X1 [KO:K01847] [EC:5.4.99.2]
107144538  Dld; dihydrolipoyl dehydrogenase, mitochondrial isoform X1 [KO:K00382] [EC:1.8.1.4]
107145494  [KO:K07513] [EC:2.3.1.16]
107145495  [KO:K07513] [EC:2.3.1.16]
107145499  [KO:K07513] [EC:2.3.1.16]
107145885  Acat1; acetyl-CoA acetyltransferase, mitochondrial [KO:K00626] [EC:2.3.1.9]
107146132  Hibadh; 3-hydroxyisobutyrate dehydrogenase, mitochondrial [KO:K00020] [EC:1.1.1.31]
107146393  Oxct1; succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial [KO:K01027] [EC:2.8.3.5]
107146423  Acadm; medium-chain specific acyl-CoA dehydrogenase, mitochondrial isoform X1 [KO:K00249] [EC:1.3.8.7]
107146635  Abat; 4-aminobutyrate aminotransferase, mitochondrial [KO:K13524] [EC:2.6.1.19 2.6.1.22]
107147436  Aldh9a1; 4-trimethylaminobutyraldehyde dehydrogenase [KO:K00149] [EC:1.2.1.47 1.2.1.3]
107147775  Aldh1b1; aldehyde dehydrogenase X, mitochondrial [KO:K00128] [EC:1.2.1.3]
107148484  Mcee; methylmalonyl-CoA epimerase, mitochondrial isoform X1 [KO:K05606] [EC:5.1.99.1]
107149161  Aldh6a1; methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial isoform X1 [KO:K00140] [EC:1.2.1.18 1.2.1.27]
107149444  Mccc1; methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial [KO:K01968] [EC:6.4.1.4]
107150817  Acsf3; malonate--CoA ligase ACSF3, mitochondrial isoform X3 [KO:K18660] [EC:6.2.1.76 6.2.1.-]
107151125  Agxt2; alanine--glyoxylate aminotransferase 2, mitochondrial isoform X2 [KO:K00827] [EC:2.6.1.44 2.6.1.40]
107151225  Il4i1; L-amino-acid oxidase isoform X3 [KO:K03334] [EC:1.4.3.2]
107151252  Aldh16a1; aldehyde dehydrogenase family 16 member A1 isoform X1 [KO:K00128] [EC:1.2.1.3]
107151273  Bcat2; branched-chain-amino-acid aminotransferase, mitochondrial isoform X1 [KO:K00826] [EC:2.6.1.42]
107151423  Bckdha; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial [KO:K00166] [EC:1.2.4.4]
107151940  Hmgcs1; hydroxymethylglutaryl-CoA synthase, cytoplasmic [KO:K01641] [EC:2.3.3.10]
107152295  Mccc2; methylcrotonoyl-CoA carboxylase beta chain, mitochondrial isoform X1 [KO:K01969] [EC:6.4.1.4]
107152775  Bcat1; branched-chain-amino-acid aminotransferase, cytosolic isoform X1 [KO:K00826] [EC:2.6.1.42]
107152844  Acadsb; short/branched chain specific acyl-CoA dehydrogenase, mitochondrial isoform X1 [KO:K09478] [EC:1.3.8.5]
107152881  Echs1; enoyl-CoA hydratase, mitochondrial isoform X2 [KO:K07511] [EC:4.2.1.17]
107153119  Acat2; acetyl-CoA acetyltransferase, cytosolic [KO:K00626] [EC:2.3.1.9]
107153404  Bckdhb; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial isoform X1 [KO:K00167] [EC:1.2.4.4]
107153524  Acad8; isobutyryl-CoA dehydrogenase, mitochondrial isoform X1 [KO:K11538] [EC:1.3.99.-]
107154337  [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
107154339  [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
107156212  Hmgcs2; hydroxymethylglutaryl-CoA synthase, mitochondrial [KO:K01641] [EC:2.3.3.10]
107156372  Aacs; acetoacetyl-CoA synthetase [KO:K01907] [EC:6.2.1.16]
107156443  Aldh2; aldehyde dehydrogenase, mitochondrial isoform X2 [KO:K00128] [EC:1.2.1.3]
107156526  Dbt; lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial isoform X1 [KO:K09699] [EC:2.3.1.168]
107157132  Aldh3a2; aldehyde dehydrogenase family 3 member A2 isoform X1 [KO:K00128] [EC:1.2.1.3]
107157633  Auh; methylglutaconyl-CoA hydratase, mitochondrial isoform X1 [KO:K05607] [EC:4.2.1.18]
107158698  Acads; short-chain specific acyl-CoA dehydrogenase, mitochondrial isoform X2 [KO:K00248] [EC:1.3.8.1]
107159769  [KO:K03334] [EC:1.4.3.2]
107159874  Hsd17b10; 3-hydroxyacyl-CoA dehydrogenase type-2 [KO:K08683] [EC:1.1.1.35 1.1.1.178]
125620403  [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
mmma00020  Citrate cycle (TCA cycle)
mmma00240  Pyrimidine metabolism
mmma00290  Valine, leucine and isoleucine biosynthesis
mmma00640  Propanoate metabolism
mmma00900  Terpenoid backbone biosynthesis
KO pathway
ko00280   

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