2-hydroxybutyric acid
362,296 routes · scope human · up to 3 steps · built 5 October 2026 · Reactions · API
313 compounds within 3 steps: 10 in 1, 72 in 2, 231 in 3
Three steps
- transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
- N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
- 1-hexadecanoyl-2-nonadioyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 2-nonadioyl-sn-glycero-3-phosphocholine + 1-hexadecanoyl-N-(acetyl)-sphing-4-enine
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosol
- 2-hydroxybutyrate:NAD+ oxidoreductase
- L-threonine = 2-oxobutanoate + NH4(+)
- L-threonine = acetaldehyde + glycine
Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 3 morecytosolOne of 4 routes
- transport of 2-hydroxybutyrate and formate (cytosol to extracellular)
- transport of chloride and formate (cytosol to extracellular)
- transport of chloride and choline (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
- Acetoacetate:CoA ligase (AMP-forming)
- 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosolOne of 3 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- alanine racemase
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol, mitochondrial matrix
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and leucine (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 12 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and homoserine (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 6 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (S)-malate + NAD(+) = pyruvate + CO2 + NADH
- malonate(out) + (S)-malate(in) = malonate(in) + (S)-malate(out)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes
- transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
- (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
- AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 1 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes
- transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
- N-acetyl-D-glucosamine 6-phosphate + H2O = D-glucosamine 6-phosphate + acetate
- N-Acetyl-D-glucosamine-6-phosphate amidohydrolase
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and histidine (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 5 routes
- transport of 2-hydroxybutyrate and L-lactate (cytosol to extracellular)
- (S)-Lactate:ferricytochrome-c 2-oxidoreductase
- 13(S)-HPODE to 13-oxy-radical-octadecadienoate conversion
Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A3 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 4 routes
- transport of 2-hydroxybutyrate and retinoate (cytosol to extracellular)
- retinoate reduction
- all-trans-Retinal 11-cis-trans-isomerase
Catalysed by SLC16A4, SLC16A5, SLC16A8, SLC16A1 and 2 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- pyruvate kinase (dTDP)
- nucleoside-diphosphate kinase (dADP)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 10 moreextracellular space, cytosolOne of 3 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (S)-malate + NAD(+) = pyruvate + CO2 + NADH
- transport of malate and sulfate (cytosol to mitochondria)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 5 routes
- transport of 2-hydroxybutyrate and L-lactate (cytosol to extracellular)
- transport of L-lactate and urate (cytosol to extracellular)
- Peroxidase
Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A3 and 5 moreextracellular space, cytosol, nucleusOne of 3 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 5 moreextracellular space, cytosol, mitochondrial matrix
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- pyruvate kinase (dTDP)
- deoxythymidylate kinase (dADP)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 5 moreextracellular space, cytosol, mitochondrial matrixOne of 3 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 3 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and citrulline (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 4 routes
- transport of 2-hydroxybutyrate and formate (cytosol to extracellular)
- transport of chloride and formate (cytosol to extracellular)
- transport of chloride and iodide (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 1 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and valine (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 11 routes
- transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
- cytochrome P450 (17alpha-hydroxyprogesterone)
- (17R,20S)-17,20-dihydroxypregn-4-en-3-one + NAD(+) = 17alpha-hydroxyprogesterone + NADH + H(+)
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosol, endoplasmic reticulumOne of 2 routes
- transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
- cytochrome P450 (17alpha-hydroxyprogesterone)
- cytochrome P450 (17alpha-hydroxyprogesterone)
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 1 moreextracellular space, cytosol, endoplasmic reticulumOne of 5 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and arginine (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 10 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- pyruvate kinase (dTDP)
- dGTP:pyruvate 2-O-phosphotransferase
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- PROTEIN_BS
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes
- 2-hydroxybutyrate:NAD+ oxidoreductase
- Selenocystathionine Lysteine-lyase (deaminating)
- Selenocystathionine Lysteine-lyase (deaminating)
Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 1 morecytosolOne of 2 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
- L-Methionine:tRNAMet ligase (AMP-forming)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 5 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- L-Serine:pyruvate aminotransferase
- D-glycerate:NADP+ 2-oxidoreductase
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosolOne of 4 routes
- 2-hydroxybutyrate:NAD+ oxidoreductase
- (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
- glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 2 morecytosolOne of 2 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
- (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 8 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and L-lactate (cytosol to extracellular)
- Transport of Prostaglandin A2, Lactate Antiport
- transport of GSH and prostaglandin A2 (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A3 and 4 moreextracellular space, cytosolOne of 4 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and threonine (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 8 routes
- transport of 2-hydroxybutyrate and formate (cytosol to extracellular)
- transport of chloride and formate (cytosol to extracellular)
- transport of chloride and oxalate (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- L-Serine:pyruvate aminotransferase
- hydroxypyruvate carboxy-lyase (glycolaldehyde-forming)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 2 routes
- transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
- 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
- 4-maleylacetoacetate = 4-fumarylacetoacetate
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
- N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 2 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and isoleucine (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 8 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and proline (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 8 routes
- transport of 2-hydroxybutyrate and L-lactate (cytosol to extracellular)
- transport of L-lactate and urate (cytosol to extracellular)
- ascorbate to monodehydroascorbate conversion
Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A3 and 4 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
- 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)
- 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 3 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
- (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
- 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 3 moreextracellular space, cytosolOne of 3 routes
- transport of 2-hydroxybutyrate and retinoate (cytosol to extracellular)
- retinoate reduction
- all-trans-retinol + NADP(+) = all-trans-retinal + NADPH + H(+)
Catalysed by SLC16A4, SLC16A5, SLC16A8, SLC16A1 and 11 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (S)-malate + NAD(+) = pyruvate + CO2 + NADH
- transport of malate and sulfite (cytosol to mitochondria)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (S)-malate + NAD(+) = pyruvate + CO2 + NADH
- (S)-malate + a quinone = enol-oxaloacetate + a quinol
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 5 moreextracellular space, cytosol
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 7 routes
- transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
- (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
- transport of alanine and cysteine (cytosol to extracellular)
Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 23 routes