2-hydroxybutyric acid

Three steps

• glutarateReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  3. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 2 morecytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrix

• nicotinateReach from here →
  1. transport of 2-hydroxybutyrate and L-lactate (cytosol to extracellular)
  2. transport of L-lactate and urate (cytosol to extracellular)
  3. nicotinate(in) + urate(out) = nicotinate(out) + urate(in)

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A3 and 5 moreextracellular space, cytosol, inner side of the membrane, outer side of the membrane

• putrescineReach from here →
  1. transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
  2. N-acetylputrescine + H2O = putrescine + acetate
  3. N-acetylputrescine + H2O = putrescine + acetate

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 1 moreextracellular space, cytosol

• cis-aconitic acidReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. pyruvate kinase (dTDP)
  3. transport of cis-aconitate and PEP (cytosol to mitochondria)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 5 moreextracellular space, cytosol, mitochondrial matrixOne of 5 routes

• thiosulfateReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. transport of malate and thiosulfate (cytosol to mitochondria)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 4 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 5 moreextracellular space, cytosol

• a phenolReach from here →
  1. transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
  2. a phenyl acetate + H2O = a phenol + acetate + H(+)
  3. a phenyl acetate + H2O = a phenol + acetate + H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 3 moreextracellular space, cytosol

• 2-oxohexanoateReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 1 morecytosol

• D-cysteineReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• all-trans-retinoateReach from here →
  1. transport of 2-hydroxybutyrate and retinoate (cytosol to extracellular)
  2. retinoate reduction
  3. all-trans-retinal + NAD(+) + H2O = all-trans-retinoate + NADH + 2 H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A8, SLC16A1 and 5 moreextracellular space, cytosolOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 2 morecytosol

• (R)-mevalonateReach from here →
  1. transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
  2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  3. (R)-mevalonate + 2 NADP(+) + CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + 2 NADPH + 2 H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 3 moreextracellular space, cytosol

• 13-cis-retinolReach from here →
  1. transport of 2-hydroxybutyrate and retinoate (cytosol to extracellular)
  2. retinal dehydrogenase (13-cis-retinal)
  3. retinol dehydrogenase (13-cis-retinal)

  Catalysed by SLC16A4, SLC16A5, SLC16A8, SLC16A1 and 3 moreextracellular space, cytosol, endoplasmic reticulumOne of 3 routes

• L-ornithineReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and ornithine (cytosol to extracellular)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 8 routes

• gamma-Glu-AlaReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. gamma-glutamyltransferase (alanine)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 9 moreextracellular space, cytosol, mitochondrial matrix

• taurineReach from here →
  1. transport of 2-hydroxybutyrate and formate (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosolOne of 2 routes

• succinyl-CoAReach from here →
  1. transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
  2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  3. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 3 moreextracellular space, cytosol

• glycineReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. L-threonine = 2-oxobutanoate + NH4(+)
  3. L-threonine = acetaldehyde + glycine

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 3 morecytosolOne of 34 routes

• (3S)-3-hydroxybutanoyl-CoAReach from here →
  1. transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
  2. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate
  3. (3S)-3-hydroxybutanoyl-CoA + NAD(+) = acetoacetyl-CoA + NADH + H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 3 moreextracellular space, cytosol

• (S)-malyl-CoAReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 5 moreextracellular space, cytosol

• (R)-methylmalonyl-CoAReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrix

• (S)-methylmalonyl-CoAReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. 2-oxobutyrate oxidation
  3. propanoyl-CoA:carbon-dioxide ligase (ADP-forming)

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 2 morecytosol, mitochondrial matrix

• (2E)-butenoyl-CoAReach from here →
  1. transport of 2-hydroxybutyrate and butyrate (cytosol to extracellular)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 8 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• acryloyl-CoAReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. 2-oxobutyrate oxidation
  3. medium-chain acyl-CoA dehydrogenase (propanoyl-CoA)

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 2 morecytosol, mitochondrial matrix

• glutaryl-CoAReach from here →
  1. transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
  2. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  3. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 3 moreextracellular space, cytosol

• propanoyl-CoAReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine
  3. 2-methyl-3-oxopropanoate + NAD(+) + CoA + H2O = propanoyl-CoA + hydrogencarbonate + NADH + H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol

• prostaglandin E1Reach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin E1(out) + 2 (S)-lactate(in) = prostaglandin E1(in) + 2 (S)-lactate(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 8 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin I2Reach from here →
  1. transport of 2-hydroxybutyrate and L-lactate (cytosol to extracellular)
  2. Transport of 15-Keto-Prostaglandin F2A, Lactate Antiport
  3. Formation of Prostaglandin I2

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A3 and 3 moreextracellular space, cytosol

• prostaglandin F2alphaReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin F2alpha(out) + 2 (S)-lactate(in) = prostaglandin F2alpha(in) + 2 (S)-lactate(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 8 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• prostaglandin H2Reach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin H2(out) + 2 (S)-lactate(in) = prostaglandin H2(in) + 2 (S)-lactate(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 8 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin D2Reach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin D2(out) + 2 (S)-lactate(in) = prostaglandin D2(in) + 2 (S)-lactate(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 8 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• D-alanineReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• (6R)-5,10-methenyltetrahydrofolateReach from here →
  1. transport of 2-hydroxybutyrate and formate (cytosol to extracellular)
  2. Formate:tetrahydrofolate ligase (ADP-forming)
  3. 5,10-Methenyltetrahydrofolate 5-hydrolase (decyclizing)

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 1 moreextracellular space, cytosolOne of 2 routes

• (13S)-hydroperoxy-(9Z,11E)-octadecadienoateReach from here →
  1. transport of 2-hydroxybutyrate and L-lactate (cytosol to extracellular)
  2. (S)-Lactate:ferricytochrome-c 2-oxidoreductase
  3. 13(S)-HPODE to 13-oxy-radical-octadecadienoate conversion

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A3 and 4 moreextracellular space, cytosol, mitochondrial matrix

• 2-oxoadipateReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-2-aminoadipate + glyoxylate = 2-oxoadipate + glycine

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 2 morecytosol

• guanidinoacetateReach from here →
  1. transport of 2-hydroxybutyrate and formate (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. Transport of Guanidinoacetic Acid

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 1 moreextracellular space, cytosol

• sphing-4-enineReach from here →
  1. transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 1 moreextracellular space, cytosol

• D-threonineReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-acetyl-alpha-D-glucosamine 1-phosphateReach from here →
  1. transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
  2. N-acetyl-D-glucosamine 6-phosphate + H2O = D-glucosamine 6-phosphate + acetate
  3. N-acetyl-alpha-D-glucosamine 1-phosphate = N-acetyl-D-glucosamine 6-phosphate

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosol

• CMP-N-acetyl-beta-neuraminateReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. CTP:N-acetylneuraminate cytidylyltransferase

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, nucleusOne of 2 routes

• spermidineReach from here →
  1. transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
  2. N(8)-acetylspermidine + H2O = spermidine + acetate
  3. N8-acetylspermidine to spermidine conversion

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 1 moreextracellular space, cytosolOne of 2 routes

• a 2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. N-(acetyl)-sphing-4-enine + a 1,2-diacyl-sn-glycero-3-phosphocholine = 1-O-acyl-N-(acetyl)-sphing-4-enine + a 2-acyl-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A1 and 2 moreextracellular space, cytosol

• L-tryptophanReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and tryptophan (cytosol to extracellular)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 10 routes

• glutathioneReach from here →
  1. transport of 2-hydroxybutyrate and L-lactate (cytosol to extracellular)
  2. transport of L-lactate and urate (cytosol to extracellular)
  3. urate(out) + glutathione(in) = urate(in) + glutathione(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A3 and 5 moreextracellular space, cytosol, inner side of the membrane, outer side of the membrane

• L-kynurenineReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrix

• beta-alanineReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 4 routes

• 2-hydroxy-3-oxopropanoateReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate aldose-ketose-isomerase

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosolOne of 2 routes

• L-asparagineReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and asparagine (cytosol to extracellular)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 11 routes

• L-phenylalanineReach from here →
  1. transport of 2-hydroxybutyrate and pyruvate (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and phenylalanine (cytosol to extracellular)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 14 routes