acetoacetate

Three steps

• acetaldehydeReach 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 3 routes

• cholineReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Acetyl-CoA:choline O-acetyltransferase

  Catalysed by AACS, HMGCS1, CHATcytosol, nucleusOne of 2 routes

• CHEBI:15380Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by AACS, HMGCS1, ALAS1, CPNE7cytosol, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by AACS, HMGCS1, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Production of Tiglylcarnitine

  Catalysed by AACS, HMGCS1, CPT1A, CPT1BcytosolOne of 2 routes

• isobutyryl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by AACS, HMGCS1, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

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

  Catalysed by AACS, HMGCS1, GLYATcytosol, mitochondrial matrix

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

  Catalysed by AACS, HMGCS1, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

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

  Catalysed by AACS, HMGCS1, SLC27A2, ACSL4 and 6 morecytosolOne of 11 routes

• CHEBI:15519Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. propanoyl-CoA C-acyltransferase

  Catalysed by AACS, HMGCS1, ACAA1, SCP2 and 1 morecytosol, peroxisomeOne of 2 routes

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

  Catalysed by AACS, HMGCS1, SLC27A2, ACSL4 and 6 morecytosolOne of 11 routes

• oleoyl-CoAReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by AACS, HMGCS1, CPT1A, CPT1C and 1 morecytosolOne of 11 routes

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

  Catalysed by AACS, HMGCS1cytosolOne of 3 routes

• CHEBI:15570Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. alanine racemase

  Catalysed by AGXT2mitochondrial matrix, cytosol

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

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, cytosol, extracellular spaceOne of 12 routes

• CHEBI:15650Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by AACS, HMGCS1, ZDHHC11B, NAA10 and 30 morecytosolOne of 2 routes

• CHEBI:15699Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and homoserine (cytosol to extracellular)

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, cytosol, extracellular spaceOne of 6 routes

• CHEBI:15727Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by AACS, HMGCS1, NAA10cytosol

• CHEBI:15746Reach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by AACS, HMGCS1, SAT1, SAT2cytosolOne of 2 routes

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

  Catalysed by ME2, SLC25A10mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

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

• CHEBI:15971Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and histidine (cytosol to extracellular)

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

• ferricytochrome cReach from here →
  1. transport of acetoacetate 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, SLC16A7, SLC16A8 and 2 moreextracellular space, cytosol, mitochondrial matrixOne of 4 routes

• 11-cis-retinalReach from here →
  1. transport of acetoacetate and retinoate (cytosol to extracellular)
  2. retinoate reduction
  3. all-trans-Retinal 11-cis-trans-isomerase

  Catalysed by SLC16A4, SLC16A5, RBP1, RBP2extracellular space, cytosol

• CHEBI:16174Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. pyruvate kinase (dTDP)
  3. nucleoside-diphosphate kinase (dADP)

  Catalysed by PKM, PKLR, NME4, NME6 and 4 moremitochondrial matrix, cytosolOne of 3 routes

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

  Catalysed by ME2, SLC25A10mitochondrial matrix, cytosolOne of 7 routes

• ureaReach from here →
  1. transport of acetoacetate and L-lactate (cytosol to extracellular)
  2. transport of L-lactate and urate (cytosol to extracellular)
  3. Peroxidase

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 3 moreextracellular space, cytosol, nucleusOne of 3 routes

• L-cystineReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter

  Catalysed by AGXT2, SLC7A9, SLC3A1mitochondrial matrix, cytosol, extracellular space

• CHEBI:16284Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. pyruvate kinase (dTDP)
  3. deoxythymidylate kinase (dADP)

  Catalysed by PKM, PKLR, DTYMKmitochondrial matrix, cytosolOne of 3 routes

• 11-cis-retinolReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by AACS, HMGCS1, DGAT1, LRAT and 3 morecytosol

• (R)-3-aminoisobutyric acidReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)

  Catalysed by AGXT2, ABATmitochondrial matrix, cytosolOne of 3 routes

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

  Catalysed by AGXT2, SLC7A6mitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

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

  Catalysed by SLC16A4, SLC16A5, SLC26A4extracellular space, cytosol

• ubiquinonesReach from here →
  1. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)
  2. 4-fumarylacetoacetate fumarylhydrolase
  3. succinate:quinone oxidoreductase

  Catalysed by FAH, SDHD, SDHB, SDHA and 1 morecytosol, mitochondrial matrix

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

  Catalysed by AGXT2, SLC7A5mitochondrial matrix, cytosol, extracellular spaceOne of 11 routes

• (17R,20S)-17,20-dihydroxypregn-4-en-3-oneReach from here →
  1. transport of acetate and acetoacetate (cytosol to extracellular)
  2. cytochrome P450 (17alpha-hydroxyprogesterone)
  3. (17R,20S)-17,20-dihydroxypregn-4-en-3-one + NAD(+) = 17alpha-hydroxyprogesterone + NADH + H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A6, SLC16A7 and 3 moreextracellular space, cytosol, endoplasmic reticulumOne of 2 routes

• androst-4-ene-3,17-dioneReach 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 2 moreextracellular space, cytosol, endoplasmic reticulumOne of 5 routes

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

  Catalysed by AGXT2, SLC7A6mitochondrial matrix, cytosol, extracellular spaceOne of 10 routes

• CHEBI:16497Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. pyruvate kinase (dTDP)
  3. dGTP:pyruvate 2-O-phosphotransferase

  Catalysed by PKM, PKLRmitochondrial matrix, cytosol

• protein polypeptide chainReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. PROTEIN_BS

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:16633Reach 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

• Met-tRNA(Met)Reach 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:tRNAMet ligase (AMP-forming)

  Catalysed by TYW1B, TYW1, MARS1mitochondrial matrix, cytosol

• (R)-glycerateReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. L-Serine:pyruvate aminotransferase
  3. D-glycerate:NADP+ 2-oxidoreductase

  Catalysed by AGXT, GRHPRmitochondrial matrix, cytosolOne of 4 routes

• 4-methylsulfanyl-2-oxobutanoateReach 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. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by TYW1B, TYW1, AADATmitochondrial matrix, cytosol

• 4-hydroxybutanoateReach from here →
  1. (S)-3-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + acetoacetate
  2. AKG[m] + (S)-3-hydroxybutanoate[m] = acetoacetate[m] + (R)-2-hydroxyglutarate[m]
  3. 4-hydroxybutanoate + 2-oxoglutarate = (R)-2-hydroxyglutarate + succinate semialdehyde

  Catalysed by ADHFE1mitochondrial matrix

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

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

• CHEBI:16856Reach from here →
  1. transport of acetoacetate and L-lactate (cytosol to extracellular)
  2. Transport of Prostaglandin A2, Lactate Antiport
  3. transport of GSH and prostaglandin A2 (cytosol to extracellular)

  Catalysed by SLC16A4, SLC16A5, SLC16A7, SLC16A8 and 2 moreextracellular space, cytosolOne of 4 routes

• CHEBI:16857Reach from here →
  1. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. transport of alanine and threonine (cytosol to extracellular)

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

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

  Catalysed by SLC16A4, SLC16A5, SLC26A4, SLC26A6extracellular space, cytosol

• propane-1,2-diolReach from here →
  1. Acetoacetate carboxy-lyase (acetone-forming)
  2. cytochrome P450 (acetone)
  3. aldose reductase (hydroxyacetone)

  Catalysed by CYP2E1, AKR7A2, AKR1A1, AKR1B1mitochondrial matrix, cytosol