acetoacetate

Three steps

• CHEBI:240943Reach from here →
  1. transport of acetoacetate and formate (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. 2-Methylbutyrylglycine Transport, Extracellular

  Catalysed by SLC16A4, SLC16A5, SLC26A4, SLC6A5extracellular space, cytosolOne of 2 routes

• a quinolReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by ME2, SDHB, SDHAmitochondrial matrix, cytosol

• hypochlorous acidReach from here →
  1. transport of acetoacetate and formate (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. Peroxidase

  Catalysed by SLC16A4, SLC16A5, SLC26A4, LPOextracellular space, cytosolOne of 4 routes

• potassium atomReach from here →
  1. transport of acetoacetate and formate (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of chloride (cytosol to extracellular)

  Catalysed by SLC16A4, SLC16A5, SLC26A4, SLC12A2 and 1 moreextracellular space, cytosolOne of 2 routes

• CHEBI:27540Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by AACS, HMGCS1, ACSM4, ACSM1 and 4 morecytosolOne of 6 routes

• selenomethionineReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by AGXT2, CTHmitochondrial matrix, cytosol

• CHEBI:27760Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by AGXT2, CTHmitochondrial matrix, cytosol

• 17alpha,21-dihydroxypregnenoloneReach from here →
  1. transport of acetate and acetoacetate (cytosol to extracellular)
  2. cytochrome P450 (17alpha-hydroxypregnenolone)
  3. cytochrome P450

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A7 and 3 moreextracellular space, cytosol, endoplasmic reticulum

• elaidic acidReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (elaidate)

  Catalysed by AACS, HMGCS1, SLC27A2, ACSL4 and 6 morecytosolOne of 5 routes

• 11-deoxycortisolReach from here →
  1. transport of acetate and acetoacetate (cytosol to extracellular)
  2. cytochrome P450 (17alpha-hydroxyprogesterone)
  3. cytochrome P450 (17alpha-hydroxyprogesterone)

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A7 and 3 moreextracellular space, cytosol, endoplasmic reticulumOne of 3 routes

• 3-mercaptolactic acidReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)
  3. 3-Mercaptolactate:NAD+ oxidoreductase

  Catalysed by MPST, LDHB, LDHC, LDHAL6A and 1 moremitochondrial matrix, cytosol

• 1-(9Z-octadecenoyl)-sn-glycero-3-phosphocholineReach from here →
  1. transport of acetate and acetoacetate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1,2-di-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-(9Z-octadecenoyl)-N-(acetyl)-sphing-4-enine + 1-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A7 and 3 moreextracellular space, cytosolOne of 3 routes

• 2-oxopentanoateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by AGXT2mitochondrial matrix, cytosol

• 3beta-hydroxyandrost-5-en-17-oneReach from here →
  1. transport of acetate and acetoacetate (cytosol to extracellular)
  2. cytochrome P450 (17alpha-hydroxypregnenolone)
  3. cytochrome P450 (17alpha-hydroxypregnenolone)

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A7 and 2 moreextracellular space, cytosol, endoplasmic reticulumOne of 6 routes

• CHEBI:28701Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by AACS, HMGCS1, ACAA1, SCP2 and 1 morecytosol, peroxisomeOne of 7 routes

• pyridoxine 5'-phosphateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by AGXT2, SCLYmitochondrial matrix, cytosol

• a dicarboxylateReach from here →
  1. transport of acetoacetate and L-lactate (cytosol to extracellular)
  2. transport of L-lactate and urate (cytosol to extracellular)
  3. urate(out) + a dicarboxylate(in) = urate(in) + a dicarboxylate(out)

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 3 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-glycoloylneuraminateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. CMP-N-acetylneuraminate monooxygenase (N-acetylneuraminate)

  Catalysed by NPLmitochondrial matrix, cytosol

• a carboxylateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by FAHD1mitochondrial matrix, cytosol

• hydrogen sulfideReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

  Catalysed by MPSTmitochondrial matrix, cytosol

• L-aspartateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. oxaloacetate + L-alanine = L-aspartate + pyruvate

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 8 routes

• citric acidReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)

  Catalysed by PC, CSmitochondrial matrix, cytosolOne of 9 routes

• malonic acidReach from here →
  1. transport of acetoacetate and formate (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

  Catalysed by SLC16A4, SLC16A5, SLC26A4extracellular space, cytosolOne of 2 routes

• isocitric acidReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. pyruvate kinase (dTDP)
  3. transport of isocitrate and PEP (cytosol to mitochondria)

  Catalysed by PKM, PKLR, SLC25A1mitochondrial matrix, cytosolOne of 5 routes

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

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 3 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 8 routes

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

  Catalysed by PC, GOT2mitochondrial matrix, cytosol

• nicotinateReach from here →
  1. transport of acetoacetate 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, SLC16A7, SLC16A8 and 3 moreextracellular space, cytosol, inner side of the membrane, outer side of the membrane

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

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A7 and 2 moreextracellular space, cytosol

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

  Catalysed by PKM, PKLR, SLC25A1mitochondrial matrix, cytosolOne of 5 routes

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

  Catalysed by ME2, SLC25A10mitochondrial matrix, cytosolOne of 6 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  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 TYW1B, TYW1, IL4I1mitochondrial matrix, cytosol

• a phenolReach from here →
  1. transport of acetate and acetoacetate (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, SLC16A6, SLC16A7 and 4 moreextracellular space, cytosol

• 2-oxohexanoateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by AGXT2mitochondrial matrix, cytosol

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

  Catalysed by AGXT2, SLC7A10mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by SLC16A4, SLC16A5, ALDH1A2, ALDH1A1 and 3 moreextracellular space, cytosolOne of 2 routes

• L-alpha-aminobutyric acidReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by AGXT2, GPTmitochondrial matrix, cytosol

• 5-aminolevulinateReach from here →
  1. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA
  2. 3-hydroxy-3-methylglutarate + succinyl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + succinate
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by HMGCL, HMGCLL1, SUGCT, ALAS1 and 1 more

• glyoxylateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)

  Catalysed by ME2, CLYBLmitochondrial matrix, cytosolOne of 8 routes

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

  Catalysed by SLC16A4, SLC16A5, ALDH1A2, ALDH1A1 and 1 moreextracellular space, cytosol, endoplasmic reticulumOne of 3 routes

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

  Catalysed by AGXT2, SLC7A6mitochondrial matrix, cytosol, extracellular spaceOne of 8 routes

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

  Catalysed by AGXT2, GGT1, GGT5, GGTLC2 and 3 moremitochondrial matrix, cytosol, extracellular space

• taurineReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by AACS, HMGCS1, BAATcytosolOne of 4 routes

• glycineReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. glycine N-acyltransferase (N-arachidonoylglycine)

  Catalysed by AACS, HMGCS1, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 27 routes

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

  Catalysed by ME2, SUCLG1, SUCLA2mitochondrial matrix, cytosol

• (R)-methylmalonyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by AACS, HMGCS1, ALDH6A1cytosol, mitochondrial matrixOne of 3 routes

• (S)-methylmalonyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by AACS, HMGCS1cytosol, mitochondrial matrixOne of 2 routes

• 3'-dephospho-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by AACS, HMGCS1, ACP2cytosol, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by AACS, HMGCS1cytosol, mitochondrial matrixOne of 3 routes

• (2E)-butenoyl-CoAReach from here →
  1. transport of acetoacetate 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 9 moreextracellular space, cytosol, mitochondrial matrixOne of 4 routes

• 3-methyl-(2E)-glutaconyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. AU RNA binding methylglutaconyl-CoA hydratase (3-methylglutaconyl-CoA)

  Catalysed by AACS, HMGCS1, AUHcytosol, mitochondrial matrix