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)

acetyl-CoA C-acetyltransferase [EC:2.3.1.9]

3-hydroxybutyryl-CoA dehydrogenase [EC:1.1.1.157]

3-hydroxyacyl-CoA dehydrogenase [EC:1.1.1.35]

3-hydroxyacyl-CoA dehydrogenase / enoyl-CoA hydratase / 3-hydroxybutyryl-CoA epimerase / enoyl-CoA isomerase [EC:5.3.3.8 5.1.2.3 4.2.1.17 1.1.1.35]

3-hydroxyacyl-CoA dehydrogenase / enoyl-CoA hydratase / 3-hydroxybutyryl-CoA epimerase [EC:5.1.2.3 4.2.1.17 1.1.1.35]

enoyl-CoA hydratase [EC:4.2.1.17]

enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase [EC:1.1.1.211 4.2.1.17]

enoyl-CoA hydratase / 3-hydroxyacyl-CoA dehydrogenase / 3,2-trans-enoyl-CoA isomerase [EC:5.3.3.8 1.1.1.35 4.2.1.17]

enoyl-CoA hydratase [EC:4.2.1.17]

butyryl-CoA dehydrogenase [EC:1.3.8.1]

trans-2-enoyl-CoA reductase (NAD+) [EC:1.3.1.44]

acetate CoA-transferase alpha subunit [EC:2.8.3.8]

acetate CoA-transferase beta subunit [EC:2.8.3.8]

medium-chain acyl-CoA synthetase [EC:6.2.1.2]

butyrate kinase [EC:2.7.2.7]

phosphate butyryltransferase [EC:2.3.1.19]

acetaldehyde dehydrogenase (acetylating) [EC:1.2.1.10]

acetaldehyde dehydrogenase / alcohol dehydrogenase [EC:1.1.1.1 1.2.1.10]

acetaldehyde dehydrogenase [EC:1.2.1.10]

[EC:1.1.1.-]

3-hydroxybutyryl-CoA dehydratase [EC:4.2.1.55]

polyhydroxyalkanoate synthase [EC:2.3.1.-]

poly(3-hydroxybutyrate) depolymerase [EC:3.1.1.75]

acetoacetyl-CoA reductase [EC:1.1.1.36]

glutaconyl-CoA decarboxylase [EC:4.1.1.70]

[EC:4.2.1.-]

glutaconate CoA-transferase, subunit A [EC:2.8.3.12]

glutaconate CoA-transferase, subunit B [EC:2.8.3.12]

2-hydroxyglutarate dehydrogenase [EC:1.1.99.2]

tartrate dehydrogenase/decarboxylase / D-malate dehydrogenase [EC:1.1.1.83 4.1.1.73 1.1.1.93]

maleate isomerase [EC:5.2.1.1]

succinate dehydrogenase flavoprotein subunit [EC:1.3.99.1]

succinate dehydrogenase iron-sulfur subunit [EC:1.3.99.1]

succinate dehydrogenase cytochrome b556 subunit

succinate dehydrogenase membrane anchor subunit

fumarate reductase flavoprotein subunit [EC:1.3.99.1]

fumarate reductase iron-sulfur subunit [EC:1.3.99.1]

fumarate reductase subunit C

fumarate reductase subunit D

succinate-semialdehyde dehydrogenase (NADP+) [EC:1.2.1.16]

succinate-semialdehyde dehydrogenase [EC:1.2.1.24]

4-aminobutyrate aminotransferase [EC:2.6.1.19]

4-aminobutyrate aminotransferase / (S)-3-amino-2-methylpropionate transaminase [EC:2.6.1.22 2.6.1.19]

4-aminobutyrate aminotransferase / (S)-3-amino-2-methylpropionate transaminase [EC:2.6.1.22 2.6.1.19]

4-aminobutyrate---pyruvate transaminase [EC:2.6.1.96]

glutamate decarboxylase [EC:4.1.1.15]

4-hydroxybutyrate dehydrogenase [EC:1.1.1.61]

4-hydroxybutyryl-CoA dehydratase / vinylacetyl-CoA-Delta-isomerase [EC:5.3.3.3 4.2.1.120]

formate C-acetyltransferase [EC:2.3.1.54]

pyruvate ferredoxin oxidoreductase, alpha subunit [EC:1.2.7.1]

pyruvate ferredoxin oxidoreductase, beta subunit [EC:1.2.7.1]

pyruvate ferredoxin oxidoreductase, gamma subunit [EC:1.2.7.1]

pyruvate ferredoxin oxidoreductase, delta subunit [EC:1.2.7.1]

hydroxymethylglutaryl-CoA synthase [EC:2.3.3.10]

hydroxymethylglutaryl-CoA lyase [EC:4.1.3.4]

3-oxoacid CoA-transferase [EC:2.8.3.5]

3-oxoacid CoA-transferase subunit A [EC:2.8.3.5]

3-oxoacid CoA-transferase subunit B [EC:2.8.3.5]

acetoacetyl-CoA synthetase [EC:6.2.1.16]

3-hydroxybutyrate dehydrogenase [EC:1.1.1.30]

hydroxybutyrate-dimer hydrolase [EC:3.1.1.22]

pyruvate dehydrogenase E1 component [EC:1.2.4.1]

pyruvate dehydrogenase E1 component subunit alpha [EC:1.2.4.1]

pyruvate dehydrogenase E1 component subunit beta [EC:1.2.4.1]

acetolactate synthase I/II/III large subunit [EC:2.2.1.6]

acetolactate synthase I/III small subunit [EC:2.2.1.6]

acetolactate synthase II small subunit [EC:2.2.1.6]

acetolactate decarboxylase [EC:4.1.1.5]

(R,R)-butanediol dehydrogenase / diacetyl reductase [EC:1.1.1.303 1.1.1.4]

(R,R)-butanediol dehydrogenase / diacetyl reductase [EC:1.1.1.303 1.1.1.4]

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