KEGG   PATHWAY: hsa00650Help
Entry
hsa00650                    Pathway                                

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

All organismsOrtholog table
Disease
H00179  
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
H00835  
Succinic semialdehyde dehydrogenase (SSADH) deficiency
H01097  
Cerebral palsy
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
Drug
D00399  
Valproic acid (USP)
D00535  
Vigabatrin (JAN/USAN/INN)
D00710  
Sodium valproate (JP17)
D08667  
Calcium valproate
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/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [KO:K07515] [EC:1.1.1.211 4.2.1.17]
1962  
EHHADH; enoyl-CoA, hydratase/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, mitochondrial [KO:K07511] [EC:4.2.1.17]
35  
ACADS; acyl-CoA dehydrogenase, C-2 to C-3 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, type 1 [KO:K00019] [EC:1.1.1.30]
56898  
BDH2; 3-hydroxybutyrate dehydrogenase, type 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
KO pathway
 

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