KEGG   REACTION: R01214
Entry
R01214                      Reaction                               
Name
L-valine:2-oxoglutarate aminotransferase
Definition
L-Valine + 2-Oxoglutarate <=> 3-Methyl-2-oxobutanoic acid + L-Glutamate
Equation
Reaction class
RC00006  C00025_C00026
RC00036  C00141_C00183
Enzyme
2.6.1.6         2.6.1.42
Pathway
rn00280  Valine, leucine and isoleucine degradation
rn00290  Valine, leucine and isoleucine biosynthesis
rn00770  Pantothenate and CoA biosynthesis
rn00966  Glucosinolate biosynthesis
rn01100  Metabolic pathways
rn01110  Biosynthesis of secondary metabolites
rn01210  2-Oxocarboxylic acid metabolism
rn01230  Biosynthesis of amino acids
rn01240  Biosynthesis of cofactors
Module
M00019  Valine/isoleucine biosynthesis, pyruvate => valine / 2-oxobutanoate => isoleucine
M00119  Pantothenate biosynthesis, valine/L-aspartate => pantothenate
Brite
Enzymatic reactions [BR:br08201]
 2. Transferase reactions
  2.6  Transferring nitrogenous groups
   2.6.1  Transaminases
    2.6.1.6
     R01214  L-Valine + 2-Oxoglutarate <=> 3-Methyl-2-oxobutanoic acid + L-Glutamate
    2.6.1.42
     R01214  L-Valine + 2-Oxoglutarate <=> 3-Methyl-2-oxobutanoic acid + L-Glutamate
Orthology
K00826  branched-chain amino acid aminotransferase [EC:2.6.1.42]
Other DBs
RHEA: 24816

KEGG   REACTION: R01701
Entry
R01701                      Reaction                               
Name
3-methyl-2-oxobutanoate:[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-2-methylpropanoylating)
Definition
3-Methyl-2-oxobutanoic acid + Enzyme N6-(lipoyl)lysine <=> [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2
Equation
Comment
3-methyl-2-oxobutanoate dehydrogenase complexes:3-methyl-2-oxobutanoate dehydrogenase
a part of multi-step reaction (see R01701+R02662+R07618, possibly identical to R01210)
subsequently R02662
TPP-dependent enzyme (see R07599+R07600)
Reaction class
RC00627  C15972_C15977
RC02856  C00141_C15977
Enzyme
Pathway
rn01100  Metabolic pathways
Brite
Enzymatic reactions [BR:br08201]
 1. Oxidoreductase reactions
  1.2  Acting on the aldehyde or oxo group of donors
   1.2.4  With a disulfide as acceptor
    1.2.4.4
     R01701  3-Methyl-2-oxobutanoic acid + Enzyme N6-(lipoyl)lysine <=> [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2
Orthology
K00166  2-oxoisovalerate dehydrogenase E1 component subunit alpha [EC:1.2.4.4]
K00167  2-oxoisovalerate dehydrogenase E1 component subunit beta [EC:1.2.4.4]
K11381  2-oxoisovalerate dehydrogenase E1 component [EC:1.2.4.4]
Other DBs
RHEA: 13460

KEGG   REACTION: R02662
Entry
R02662                      Reaction                               
Name
2-methylpropanoyl-CoA:enzyme N6-(dihydrolipoyl)lysine S-(2-methylpropanoyl)transferase
Definition
2-Methylpropanoyl-CoA + Enzyme N6-(dihydrolipoyl)lysine <=> CoA + [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine
Equation
Comment
3-methyl-2-oxobutanoate dehydrogenase complexes:dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
a part of multi-step reaction (see R01701+R02662+R07618, possibly identical to R01210)
following after R01701
Reaction class
RC00004  C00010_C00630  C15973_C15977
RC02870  C00630_C15977
Enzyme
Pathway
rn00280  Valine, leucine and isoleucine degradation
rn00785  Lipoic acid metabolism
rn01100  Metabolic pathways
Brite
Enzymatic reactions [BR:br08201]
 2. Transferase reactions
  2.3  Acyltransferases
   2.3.1  Transferring groups other than aminoacyl groups
    2.3.1.168
     R02662  2-Methylpropanoyl-CoA + Enzyme N6-(dihydrolipoyl)lysine <=> CoA + [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine
Orthology
K09699  2-oxoisovalerate dehydrogenase E2 component (dihydrolipoyl transacylase) [EC:2.3.1.168]
Other DBs
RHEA: 18868

KEGG   REACTION: R02661
Entry
R02661                      Reaction                               
Name
2-methylpropanoyl-CoA:(acceptor) 2,3-oxidoreductase
Definition
2-Methylpropanoyl-CoA + Acceptor <=> 2-Methylprop-2-enoyl-CoA + Reduced acceptor
Equation
Reaction class
RC00148  C00630_C03460
Enzyme
1.3.8.1         1.3.8.5         1.3.8.7         1.3.99.-
Pathway
rn00280  Valine, leucine and isoleucine degradation
rn01100  Metabolic pathways
Brite
Enzymatic reactions [BR:br08201]
 1. Oxidoreductase reactions
  1.3  Acting on the CH-CH group of donors
   1.3.8  With a flavin as acceptor
    1.3.8.1
     R02661  2-Methylpropanoyl-CoA + Acceptor <=> 2-Methylprop-2-enoyl-CoA + Reduced acceptor
    1.3.8.5
     R02661  2-Methylpropanoyl-CoA + Acceptor <=> 2-Methylprop-2-enoyl-CoA + Reduced acceptor
    1.3.8.7
     R02661  2-Methylpropanoyl-CoA + Acceptor <=> 2-Methylprop-2-enoyl-CoA + Reduced acceptor
   1.3.99  With unknown physiological acceptors
    1.3.99.-
     R02661  2-Methylpropanoyl-CoA + Acceptor <=> 2-Methylprop-2-enoyl-CoA + Reduced acceptor
Orthology
K00248  butyryl-CoA dehydrogenase [EC:1.3.8.1]
K00249  acyl-CoA dehydrogenase [EC:1.3.8.7]
K09478  short-chain 2-methylacyl-CoA dehydrogenase [EC:1.3.8.5]
K11410  short-chain 2-methylacyl-CoA dehydrogenase [EC:1.3.8.5]
K11538  isobutyryl-CoA dehydrogenase [EC:1.3.99.-]
Reference
1  [PMID:12359132]
  Authors
Nguyen TV, Andresen BS, Corydon TJ, Ghisla S, Abd-El Razik N, Mohsen AW, Cederbaum SD, Roe DS, Roe CR, Lench NJ, Vockley J
  Title
Identification of isobutyryl-CoA dehydrogenase and its deficiency in humans.
  Journal
Mol Genet Metab 77:68-79 (2002)
DOI:10.1016/S1096-7192(02)00152-X
Reference
2  [PMID:11013134]
  Authors
Andresen BS, Christensen E, Corydon TJ, Bross P, Pilgaard B, Wanders RJ, Ruiter JP, Simonsen H, Winter V, Knudsen I, Schroeder LD, Gregersen N, Skovby F
  Title
Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism.
  Journal
Am J Hum Genet 67:1095-103 (2000)
DOI:10.1086/303105
Other DBs
RHEA: 31098

KEGG   REACTION: R04224
Entry
R04224                      Reaction                               
Name
(S)-3-hydroxyisobutyryl-CoA hydro-lyase
Definition
2-Methylprop-2-enoyl-CoA + H2O <=> (S)-3-Hydroxyisobutyryl-CoA
Equation
Reaction class
RC01103  C03460_C06000
Enzyme
Pathway
rn00280  Valine, leucine and isoleucine degradation
rn01100  Metabolic pathways
Brite
Enzymatic reactions [BR:br08201]
 4. Lyase reactions
  4.2  Carbon-oxygen lyases
   4.2.1  Hydro-lyases
    4.2.1.17
     R04224  2-Methylprop-2-enoyl-CoA + H2O <=> (S)-3-Hydroxyisobutyryl-CoA
IUBMB reaction hierarchy [BR:br08202]
 4. Lyase reactions
  4.2.1.17
   R02685  (3S)-3-Hydroxyacyl-CoA <=> trans-2,3-Dehydroacyl-CoA
    R04224  (S)-3-Hydroxyisobutyryl-CoA <=> 2-Methylprop-2-enoyl-CoA
Orthology
K01692  enoyl-CoA hydratase [EC:4.2.1.17]
K01782  3-hydroxyacyl-CoA dehydrogenase / enoyl-CoA hydratase / 3-hydroxybutyryl-CoA epimerase [EC:1.1.1.35 4.2.1.17 5.1.2.3]
K01825  3-hydroxyacyl-CoA dehydrogenase / enoyl-CoA hydratase / 3-hydroxybutyryl-CoA epimerase / enoyl-CoA isomerase [EC:1.1.1.35 4.2.1.17 5.1.2.3 5.3.3.8]
K07511  enoyl-CoA hydratase [EC:4.2.1.17]
K07514  enoyl-CoA hydratase / 3-hydroxyacyl-CoA dehydrogenase / 3,2-trans-enoyl-CoA isomerase [EC:4.2.1.17 1.1.1.35 5.3.3.8]
K07515  enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase [EC:4.2.1.17 1.1.1.211]
Other DBs
RHEA: 31178

KEGG   REACTION: R05064
Entry
R05064                      Reaction                               
Name
(S)-3-hydroxyisobutyryl-CoA hydrolase
Definition
(S)-3-Hydroxyisobutyryl-CoA + H2O <=> CoA + (S)-3-Hydroxyisobutyrate
Equation
Reaction class
RC00004  C00010_C06000
RC00137  C06000_C06001
Enzyme
Pathway
rn00280  Valine, leucine and isoleucine degradation
rn01100  Metabolic pathways
Brite
Enzymatic reactions [BR:br08201]
 3. Hydrolase reactions
  3.1  Acting on ester bonds
   3.1.2  Thioester hydrolases
    3.1.2.4
     R05064  (S)-3-Hydroxyisobutyryl-CoA + H2O <=> CoA + (S)-3-Hydroxyisobutyrate
Orthology
K05605  3-hydroxyisobutyryl-CoA hydrolase [EC:3.1.2.4]
Other DBs
RHEA: 31254

KEGG   REACTION: R05066
Entry
R05066                      Reaction                               
Name
3-hydroxy-2-methylpropanoate:NAD+ oxidoreductase
Definition
(S)-3-Hydroxyisobutyrate + NAD+ <=> (S)-Methylmalonate semialdehyde + NADH + H+
Equation
Reaction class
RC00001  C00003_C00004
RC00099  C06001_C06002
Enzyme
Pathway
rn00280  Valine, leucine and isoleucine degradation
rn01100  Metabolic pathways
Brite
Enzymatic reactions [BR:br08201]
 1. Oxidoreductase reactions
  1.1  Acting on the CH-OH group of donors
   1.1.1  With NAD+ or NADP+ as acceptor
    1.1.1.31
     R05066  (S)-3-Hydroxyisobutyrate + NAD+ <=> (S)-Methylmalonate semialdehyde + NADH + H+
IUBMB reaction hierarchy [BR:br08202]
 1. Oxidoreductase reactions
  1.1.1.31
   R02047  3-Hydroxy-2-methylpropanoate <=> 2-Methyl-3-oxopropanoate
    R05066  (S)-3-Hydroxyisobutyrate <=> (S)-Methylmalonate semialdehyde
Orthology
K00020  3-hydroxyisobutyrate dehydrogenase [EC:1.1.1.31]
K23146  3-hydroxyisobutyrate/3-hydroxypropionate dehydrogenase [EC:1.1.1.31 1.1.1.59]
Other DBs
RHEA: 31278

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