acetoacetate

Three steps

• glycolaldehydeReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate carboxy-lyase (glycolaldehyde-forming)

  Catalysed by AGXTmitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by NPLmitochondrial matrix, cytosolOne of 2 routes

• methylglyoxalReach from here →
  1. Acetoacetate carboxy-lyase (acetone-forming)
  2. cytochrome P450 (acetone)
  3. cytochrome P450 (hydroxyacetone)

  Catalysed by CYP2E1mitochondrial matrix, cytosol

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

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, cytosol, extracellular spaceOne of 8 routes

• CHEBI:17203Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and proline (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, cytosol, extracellular spaceOne of 8 routes

• CHEBI:17208Reach from here →
  1. transport of acetoacetate and L-lactate (cytosol to extracellular)
  2. transport of L-lactate and urate (cytosol to extracellular)
  3. ascorbate to monodehydroascorbate conversion

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 2 moreextracellular space, cytosol

• 4-methylumbelliferoneReach from here →
  1. transport of acetate and acetoacetate (cytosol to extracellular)
  2. 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)
  3. 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A7 and 4 moreextracellular space, cytosol

• succinyl-4'-phosphopantetheineReach 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 + H2O = succinyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by HMGCL, HMGCLL1, SUGCT, NUDT19 and 2 more

• anilineReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by AACS, HMGCS1, NAT2, NAT1cytosol

• CHEBI:17312Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by AACS, HMGCS1cytosolOne of 2 routes

• all-trans-retinolReach from here →
  1. transport of acetoacetate and retinoate (cytosol to extracellular)
  2. retinoate reduction
  3. all-trans-retinol + NADP(+) = all-trans-retinal + NADPH + H(+)

  Catalysed by SLC16A4, SLC16A5, AKR1B15, AKR1B10 and 9 moreextracellular space, cytosol

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

  Catalysed by ME2, SLC25A10mitochondrial matrix, cytosolOne of 4 routes

• enol-oxaloacetateReach 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

• AH2Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by AGXT2, MOCOSmitochondrial matrix, cytosolOne of 8 routes

• L-cysteineReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by AACS, HMGCS1, NAGScytosol, mitochondrial matrixOne of 24 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by AACS, HMGCS1, HADHA, ACADVL and 1 morecytosol, mitochondrial matrix

• xanthineReach from here →
  1. transport of acetoacetate and L-lactate (cytosol to extracellular)
  2. transport of L-lactate and urate (cytosol to extracellular)
  3. xanthine + NAD(+) + H2O = urate + NADH + H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 3 moreextracellular space, cytosolOne of 2 routes

• Ala-tRNA(Ala)Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by AGXT2, AARS1mitochondrial matrix, cytosol

• glycine betaineReach from here →
  1. transport of acetoacetate and formate (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by SLC16A4, SLC16A5, SLC26A4, SLC6A12extracellular space, cytosolOne of 3 routes

• CHEBI:17895Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and tyrosine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, cytosol, extracellular spaceOne of 10 routes

• CHEBI:17927Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by AACS, HMGCS1cytosol

• butanoateReach from here →
  1. transport of acetoacetate and butyrate (cytosol to extracellular)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + H2O = butanoate + CoA + H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 13 moreextracellular space, cytosol

• 3-phenylpyruvateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. L-Glutamine:pyruvate aminotransferase
  3. 3-phenylpyruvate + L-glutamine = 2-oxoglutaramate + L-phenylalanine

  Catalysed by KYAT1mitochondrial matrix, cytosol

• CHEBI:18019Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and lysine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A6mitochondrial matrix, cytosol, extracellular spaceOne of 5 routes

• thiocyanateReach 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-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)

  Catalysed by MPST, TSTmitochondrial matrix, cytosol

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

  Catalysed by AGXT2, SLC3A2, SLC7A5, SLC7A10mitochondrial matrix, cytosol, extracellular spaceOne of 18 routes

• 5-hydroxyisourateReach from here →
  1. transport of acetoacetate and L-lactate (cytosol to extracellular)
  2. transport of L-lactate and urate (cytosol to extracellular)
  3. urate:oxygen oxidoreductase

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 2 moreextracellular space, cytosol

• dTTPReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. pyruvate kinase (dTDP)
  3. DTTP Nucleotidohydrolase

  Catalysed by PKM, PKLR, ENTPD3, ENTPD1 and 1 moremitochondrial matrix, cytosol, extracellular spaceOne of 11 routes

• CHEBI:18095Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. Transport of Hydroxy-Proline

  Catalysed by AGXT2, SLC1A4mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• N-carbamoyl-beta-alanineReach from here →
  1. transport of acetoacetate and formate (cytosol to extracellular)
  2. transport of chloride and formate (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

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

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. transport of acetoacetate and formate (cytosol to extracellular)
  2. Formate:tetrahydrofolate ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by SLC16A4, SLC16A5, MTHFD1, GARTextracellular space, cytosol

• CHEBI:18381Reach from here →
  1. transport of acetoacetate and formate (cytosol to extracellular)
  2. Formate:tetrahydrofolate ligase (ADP-forming)
  3. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by SLC16A4, SLC16A5, MTHFD1, ATICextracellular space, cytosol

• S-methylmercury-L-cysteineReach 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. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by TYW1B, TYW1, SLC7A8mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by AGXT2, BCKDHA, BCKDHBmitochondrial matrix, cytosol

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

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

• D-isoleucineReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. D-serine = pyruvate + NH4(+)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by SRR, SLC7A10mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membrane

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 3 routes

• 1-butanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. transport of acetoacetate and butyrate (cytosol to extracellular)
  2. Butanoate:CoA ligase (AMP-forming)
  3. 2-(9Z-octadecenoyl)-glycerol + butanoyl-CoA = 1-butanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 9 moreextracellular space, cytosol

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. transport of acetoacetate and formate (cytosol to extracellular)
  2. Formate:tetrahydrofolate ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:NADP+ oxidoreductase

  Catalysed by SLC16A4, SLC16A5, MTHFD1, ALDH1L1 and 1 moreextracellular space, cytosolOne of 4 routes

• O-butanoyl-(R)-carnitineReach from here →
  1. transport of acetoacetate and butyrate (cytosol to extracellular)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + (R)-carnitine = O-butanoyl-(R)-carnitine + CoA

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 8 moreextracellular space, cytosol

• (5Z)-dodecenoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by AACS, HMGCS1, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• (S)-lactoyl-AMPReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + H(+) = (S)-lactoyl-AMP + diphosphate

  Catalysed by LDHA, LDHB, LDHC, LDHAL6A and 3 moremitochondrial matrix, cytosol

• (S)-lactoyl-CoAReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + CoA = (S)-lactoyl-CoA + AMP + diphosphate

  Catalysed by LDHA, LDHB, LDHC, LDHAL6A and 2 moremitochondrial matrix, cytosol

• N-acetylneuraminate 9-phosphateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. pyruvate kinase (dTDP)
  3. N-acetylneuraminate synthase

  Catalysed by PKM, PKLR, NANSmitochondrial matrix, cytosol

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

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

• (7Z)-tetradecenoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by AACS, HMGCS1, SLC27A2, ACSL4 and 6 morecytosolOne of 8 routes

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by AACS, HMGCS1, HADHA, ACADVL and 1 morecytosol, mitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by AACS, HMGCS1, HADHA, ACADVL and 1 morecytosol, mitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by AACS, HMGCS1, ECHS1, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by AACS, HMGCS1, ECHS1, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 2 routes