KEGG   PATHWAY: hsa00650Help
Entry
hsa00650                    Pathway                                

Name
Butanoate metabolism - Homo sapiens (human)
Class
Metabolism; Carbohydrate metabolism
BRITE hierarchy
Pathway map
hsa00650  Butanoate metabolism
hsa00650

Ortholog table
Disease
H00179  3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
H00835  Succinic semialdehyde dehydrogenase (SSADH) deficiency
H01121  Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency
H01123  HMG-CoA synthase (HMGCS) deficiency
H01280  L-2-hydroxyglutaric aciduria (L2HGA)
H01364  3-Hydroxyacyl-CoA dehydrogenase deficiency
Organism
Homo sapiens (human) [GN:hsa]
Gene
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]
3033  HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]
3030  HADHA; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha [KO:K07515] [EC:1.1.1.211 4.2.1.17]
1962  EHHADH; enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [KO:K07514] [EC:5.3.3.8 1.1.1.35 4.2.1.17]
1892  ECHS1; enoyl-CoA hydratase, short chain 1 [KO:K07511] [EC:4.2.1.17]
35  ACADS; acyl-CoA dehydrogenase short chain [KO:K00248] [EC:1.3.8.1]
116285  ACSM1; acyl-CoA synthetase medium chain family member 1 [KO:K01896] [EC:6.2.1.2]
123876  ACSM2A; acyl-CoA synthetase medium chain family member 2A [KO:K01896] [EC:6.2.1.2]
341392  ACSM4; acyl-CoA synthetase medium chain family member 4 [KO:K01896] [EC:6.2.1.2]
54988  ACSM5; acyl-CoA synthetase medium chain family member 5 [KO:K01896] [EC:6.2.1.2]
6296  ACSM3; acyl-CoA synthetase medium chain family member 3 [KO:K01896] [EC:6.2.1.2]
348158  ACSM2B; acyl-CoA synthetase medium chain family member 2B [KO:K01896] [EC:6.2.1.2]
142827  ACSM6; acyl-CoA synthetase medium chain family member 6 [KO:K01896] [EC:6.2.1.2]
79944  L2HGDH; L-2-hydroxyglutarate dehydrogenase [KO:K00109] [EC:1.1.99.2]
2571  GAD1; glutamate decarboxylase 1 [KO:K01580] [EC:4.1.1.15]
2572  GAD2; glutamate decarboxylase 2 [KO:K01580] [EC:4.1.1.15]
18  ABAT; 4-aminobutyrate aminotransferase [KO:K13524] [EC:2.6.1.22 2.6.1.19]
7915  ALDH5A1; aldehyde dehydrogenase 5 family member A1 [KO:K00139] [EC:1.2.1.24]
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]
3155  HMGCL; 3-hydroxymethyl-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]
622  BDH1; 3-hydroxybutyrate dehydrogenase 1 [KO:K00019] [EC:1.1.1.30]
56898  BDH2; 3-hydroxybutyrate dehydrogenase 2 [KO:K00019] [EC:1.1.1.30]
Compound
C00022  Pyruvate
C00024  Acetyl-CoA
C00025  L-Glutamate
C00026  2-Oxoglutarate
C00042  Succinate
C00091  Succinyl-CoA
C00122  Fumarate
C00136  Butanoyl-CoA
C00164  Acetoacetate
C00232  Succinate semialdehyde
C00246  Butanoic acid
C00332  Acetoacetyl-CoA
C00334  4-Aminobutanoate
C00356  (S)-3-Hydroxy-3-methylglutaryl-CoA
C00497  (R)-Malate
C00741  Diacetyl
C00810  (R)-Acetoin
C00877  Crotonoyl-CoA
C00989  4-Hydroxybutanoic acid
C01089  (R)-3-Hydroxybutanoate
C01144  (S)-3-Hydroxybutanoyl-CoA
C01384  Maleic acid
C01412  Butanal
C01769  (S)-Acetoin
C02331  Vinylacetyl-CoA
C02411  Glutaconyl-1-CoA
C02527  Butanoylphosphate
C02630  2-Hydroxyglutarate
C03044  (R,R)-Butane-2,3-diol
C03046  (S,S)-Butane-2,3-diol
C03058  2-Hydroxyglutaryl-CoA
C03561  (R)-3-Hydroxybutanoyl-CoA
C04546  (R)-3-((R)-3-Hydroxybutanoyloxy)butanoate
C06010  (S)-2-Acetolactate
C06142  1-Butanol
C06143  Poly-beta-hydroxybutyrate
C06144  3-Butynoate
C06145  3-Butyn-1-al
C06146  3-Butyn-1-ol
C11062  4-Hydroxybutyryl-CoA
C20657  meso-2,3-Butanediol
C21390  Butane
KO pathway
ko00650   

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