Butanoate metabolism

3-Hydroxy-3-methylglutaryl-CoA lyase deficiency

Succinic semialdehyde dehydrogenase (SSADH) deficiency

Cerebral palsy

Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency

HMG-CoA synthase (HMGCS) deficiency

L-2-hydroxyglutaric aciduria (L2HGA)

Valproic acid (USP)

Vigabatrin (JAN/USAN/INN)

Sodium valproate (JP16)

Calcium valproate

ACAT2; acetyl-CoA acetyltransferase 2 [KO:K00626] [EC:2.3.1.9]

ACAT1; acetyl-CoA acetyltransferase 1 [KO:K00626] [EC:2.3.1.9]

HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]

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]

EHHADH; enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase [KO:K07514] [EC:5.3.3.8 1.1.1.35 4.2.1.17]

ECHS1; enoyl CoA hydratase, short chain, 1, mitochondrial [KO:K07511] [EC:4.2.1.17]

ACADS; acyl-CoA dehydrogenase, C-2 to C-3 short chain [KO:K00248] [EC:1.3.8.1]

ACSM1; acyl-CoA synthetase medium-chain family member 1 [KO:K01896] [EC:6.2.1.2]

ACSM2A; acyl-CoA synthetase medium-chain family member 2A [KO:K01896] [EC:6.2.1.2]

ACSM4; acyl-CoA synthetase medium-chain family member 4 [KO:K01896] [EC:6.2.1.2]

ACSM5; acyl-CoA synthetase medium-chain family member 5 [KO:K01896] [EC:6.2.1.2]

ACSM3; acyl-CoA synthetase medium-chain family member 3 [KO:K01896] [EC:6.2.1.2]

ACSM2B; acyl-CoA synthetase medium-chain family member 2B [KO:K01896] [EC:6.2.1.2]

L2HGDH; L-2-hydroxyglutarate dehydrogenase [KO:K00109] [EC:1.1.99.2]

ALDH5A1; aldehyde dehydrogenase 5 family, member A1 [KO:K00139] [EC:1.2.1.24]

ABAT; 4-aminobutyrate aminotransferase [KO:K13524] [EC:2.6.1.22 2.6.1.19]

GAD1; glutamate decarboxylase 1 (brain, 67kDa) [KO:K01580] [EC:4.1.1.15]

GAD2; glutamate decarboxylase 2 (pancreatic islets and brain, 65kDa) [KO:K01580] [EC:4.1.1.15]

HMGCS1; 3-hydroxy-3-methylglutaryl-CoA synthase 1 (soluble) [KO:K01641] [EC:2.3.3.10]

HMGCS2; 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) [KO:K01641] [EC:2.3.3.10]

HMGCL; 3-hydroxymethyl-3-methylglutaryl-CoA lyase [KO:K01640] [EC:4.1.3.4]

OXCT1; 3-oxoacid CoA transferase 1 [KO:K01027] [EC:2.8.3.5]

OXCT2; 3-oxoacid CoA transferase 2 [KO:K01027] [EC:2.8.3.5]

AACS; acetoacetyl-CoA synthetase [KO:K01907] [EC:6.2.1.16]

BDH1; 3-hydroxybutyrate dehydrogenase, type 1 [KO:K00019] [EC:1.1.1.30]

BDH2; 3-hydroxybutyrate dehydrogenase, type 2 [KO:K00019] [EC:1.1.1.30]

PDHA2; pyruvate dehydrogenase (lipoamide) alpha 2 [KO:K00161] [EC:1.2.4.1]

PDHA1; pyruvate dehydrogenase (lipoamide) alpha 1 [KO:K00161] [EC:1.2.4.1]

PDHB; pyruvate dehydrogenase (lipoamide) beta [KO:K00162] [EC:1.2.4.1]

Pyruvate

Acetyl-CoA

L-Glutamate

2-Oxoglutarate

Succinate

Thiamin diphosphate

Fumarate

Butanoyl-CoA

Acetoacetate

Succinate semialdehyde

Butanoic acid

Acetoacetyl-CoA

4-Aminobutanoate

(S)-3-Hydroxy-3-methylglutaryl-CoA

(R)-Malate

Diacetyl

(R)-Acetoin

Crotonoyl-CoA

2-Acetolactate

4-Hydroxybutanoic acid

(R)-3-Hydroxybutanoate

(S)-3-Hydroxybutanoyl-CoA

Maleic acid

Butanal

(S)-Acetoin

Vinylacetyl-CoA

Glutaconyl-1-CoA

Butanoylphosphate

2-Hydroxyglutarate

(R,R)-Butane-2,3-diol

(S,S)-Butane-2,3-diol

2-Hydroxyglutaryl-CoA

(R)-3-Hydroxybutanoyl-CoA

(R)-3-((R)-3-Hydroxybutanoyloxy)butanoate

2-(alpha-Hydroxyethyl)thiamine diphosphate

1-Butanol

Poly-beta-hydroxybutyrate

3-Butynoate

3-Butyn-1-al

3-Butyn-1-ol