CHEBI:1146900

Three steps

• (R)-3-hydroxybutyric acidReach from here →
  1. Propanoate:CoA ligase (AMP-forming)
  2. Propanoyl-CoA hydrolase
  3. transport of (R)-3-hydroxybutanoate and propanoate (cytosol to extracellular)

  Catalysed by ACSS3, ACSS2, ACSS1, ACOT7 and 4 morecytosol, extracellular spaceOne of 6 routes

• 3-hydroxypyruvateReach from here →
  1. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by AGXT2, AGXTmitochondrial matrixOne of 2 routes

• CHEBI:17208Reach from here →
  1. N-acetyl-LTE4 to leukotriene E4 conversion
  2. transport of GSH and leukotriene E4 (cytosol to extracellular)
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by ZDHHC11B, NAA10, ESCO2, ESCO1 and 29 morecytosol, extracellular space

• homocysteineReach from here →
  1. N-acetyl-LTE4 to leukotriene E4 conversion
  2. transport of GSH and leukotriene E4 (cytosol to extracellular)
  3. Formation of Homocystine

  Catalysed by ZDHHC11B, NAA10, ESCO2, ESCO1 and 29 morecytosol, extracellular space

• L-glutamic 5-semialdehydeReach from here →
  1. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  2. taurine + L-arginine = taurocyamine + L-ornithine
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by BAAT, GATM, OATcytosol, mitochondrial matrix

• propanoyl-4'-phosphopantetheineReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2
  3. propanoyl-CoA + H2O = propanoyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by ECHDC1, NUDT19, NUDT7, NUDT8mitochondrial matrix

• succinyl-4'-phosphopantetheineReach from here →
  1. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  2. (R)-methylmalonyl-CoA = succinyl-CoA
  3. succinyl-CoA + H2O = succinyl-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by ALDH6A1, MMUT, NUDT19, NUDT7 and 1 moremitochondrial matrix

• myo-inositolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. a 1-acyl-sn-glycero-3-phospho-(1D-myo-inositol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + CoA
  3. a CDP-1,2-diacyl-sn-glycerol + myo-inositol = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + CMP + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 moreGolgi apparatus

• propanoateReach from here →
  1. (S)-methylmalonyl-CoA hydrolase
  2. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2
  3. propanoyl-CoA + H2O = propanoate + CoA + H(+)

  Catalysed by ECHDC1, ACOT8, ACAA2, ACOT9mitochondrial matrix

• aceneuramateReach from here →
  1. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by AGXT2, NPLmitochondrial matrix

• 5'-deoxyadenosineReach from here →
  1. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  2. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)
  3. N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)

  Catalysed by BAAT, MTO1, CDK5RAP1cytosolOne of 5 routes

• 3-hydroxy-3-methylglutarateReach from here →
  1. Acetoacetate:CoA ligase (AMP-forming)
  2. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)
  3. Formation of 3-Hydroxy-3-Methyl-Glutarate

  Catalysed by AACS, HMGCS1cytosolOne of 5 routes

• sulfiteReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GSS and 1 moremitochondrial matrixOne of 4 routes

• CHEBI:17380Reach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. transport of glycine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• a 2-acylglycerolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. a 2-acylglycerol + an acyl-CoA = a 1,2-diacyl-sn-glycerol + CoA
  3. a 1,2-diacyl-sn-glycerol + H2O = a 2-acylglycerol + a fatty acid + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moreGolgi apparatus

• a monoacylglycerolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. a 2-acylglycerol + an acyl-CoA = a 1,2-diacyl-sn-glycerol + CoA
  3. 1,2-diacyl-sn-glycerol acylhydrolase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 18 moreGolgi apparatus, extracellular spaceOne of 8 routes

• agmatineReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A6mitochondrial matrix, extracellular space, cytosol

• enol-oxaloacetateReach from here →
  1. ATP citrate lyase (citrate)
  2. transport of malate and OAA (cytosol to mitochondria)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by ACLY, SDHB, SDHAcytosol, mitochondrial matrix

• O-phosphoethanolamineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. ATP:sphingosine 1-phosphotransferase
  3. sphingosine-1-phosphate palmitaldehyde-lyase (phosphoethanolamine-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moreGolgi apparatus, cytosolOne of 2 routes

• (2E)-hexadecenalReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. ATP:sphingosine 1-phosphotransferase
  3. sphingosine-1-phosphate palmitaldehyde-lyase (phosphoethanolamine-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moreGolgi apparatus, cytosolOne of 2 routes

• hexadecanalReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Utilized Transport
  3. 2 H+[c] + NADH[c] + palmitate[c] = H2O[c] + hexadecanal[c] + NAD+[c]

  Catalysed by CPT1A, CPT1C, CPT1B, ALDH3B1 and 2 morecytosol, endoplasmic reticulumOne of 2 routes

• a sphingomyelinReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. sphing-4-enine + a fatty acyl-CoA = an N-acylsphing-4-enine + CoA + H(+)
  3. ceramide:phosphatidylcholine cholinephosphotransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 moreGolgi apparatusOne of 13 routes

• indole-3-pyruvateReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• 3,3',5'-triiodo-L-thyronine sulfateReach from here →
  1. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  2. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)
  3. 3,3',5'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2

  Catalysed by BAAT, MTO1, DIO1cytosol

• 3,3'-diiodo-L-thyronineReach from here →
  1. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  2. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)
  3. 3,3'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2

  Catalysed by BAAT, MTO1, DIO1, DIO3cytosolOne of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  2. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)
  3. 3,3'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2

  Catalysed by BAAT, MTO1, DIO1cytosolOne of 2 routes

• CHEBI:17724Reach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GNMT and 2 moremitochondrial matrix, cytosol

• glycine betaineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Carnitine Transport by Atb0
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by CPT1A, CPT1C, CPT1B, SLC6A14 and 1 morecytosol, extracellular spaceOne of 7 routes

• glycerolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA
  3. a 1-acylglycerol + a 1,2-diacylglycerol = a triacylglycerol + glycerol

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 moreGolgi apparatusOne of 2 routes

• trans-urocanateReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. transport of glycine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

• urateReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. urate(out) + glutathione(in) = urate(in) + glutathione(out)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GSS and 1 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 3 routes

• 5-hydroxy-L-tryptophanReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5 and 2 moremitochondrial matrix, extracellular space, cytosol

• methylarsonous acidReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by GLYAT, GLYATL2, GLYATL1, GSS and 2 moremitochondrial matrix

• 4-methyl-2-oxopentanoateReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. transport of glycine and leucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

• all-trans-retinalReach from here →
  1. Palmitoyl-CoA:retinol O-acyltransferase
  2. all-trans-Retinol 11-cis-trans-isomerase
  3. retinol: NADP+ oxidoreductase

  Catalysed by DGAT1, LRAT, AWAT1, AWAT2 and 7 morecytosolOne of 6 routes

• 3-oxopropanoic acidReach from here →
  1. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by SLC7A10, ABATmitochondrial matrix, inner side of the membrane, outer side of the membrane

• 3-phenylpyruvateReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by GLYAT, GLYATL2, GLYATL1, SLC7A5 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 6 routes

• fumaric acidReach from here →
  1. ATP citrate lyase (citrate)
  2. transport of malate and OAA (cytosol to mitochondria)
  3. (S)-malate hydro-lyase (fumarate-forming)

  Catalysed by ACLY, FHcytosol, mitochondrial matrixOne of 5 routes

• thiocyanateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)

  Catalysed by NAGS, GOT2, GOT1, MPST and 1 moremitochondrial matrix, cytosol

• a diacylglycerolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. a triacylglycerol + H2O = a diacylglycerol + a fatty acid + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 24 moreGolgi apparatus, cytosolOne of 3 routes

• dTDPReach from here →
  1. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. pyruvate kinase (dTDP)

  Catalysed by AGXT2, PKM, PKLRmitochondrial matrix, cytosol

• dTTPReach from here →
  1. ATP citrate lyase (citrate)
  2. GTP:oxaloacetate carboxy-lyase (adding GTP
  3. pyruvate kinase (dTDP)

  Catalysed by ACLY, PCK1, PKM, PKLRcytosol

• CHEBI:18183Reach from here →
  1. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  2. gamma-glutamyltransferase (alanine)
  3. gamma-glutamylcyclotransferase ((5-L-glutamyl)-L-amino acid)

  Catalysed by GGT1, GGT5, GGTLC2, GGT6 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• N-carbamoyl-beta-alanineReach from here →
  1. Carnitine O-Palmitoyltransferase
  2. Carnitine Transport by Atb0
  3. Transport of 3-Ureidopropionate

  Catalysed by CPT1A, CPT1C, CPT1B, SLC6A14cytosol, extracellular spaceOne of 2 routes

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by GLYAT, GLYATL2, GLYATL1, GARTmitochondrial matrix, cytosol

• phosphatidyl-L-serineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase
  3. phosphatidylserine synthase (PC-LD pool)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moreGolgi apparatus, cytosolOne of 2 routes

• arsenic triglutathioneReach from here →
  1. glycine N-acyltransferase (N-arachidonoylglycine)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. arsenic triglutathione + 3 [thioredoxin]-dithiol + 3 S-adenosyl-L-methionine = trimethylarsine + 3 [thioredoxin]-disulfide + 3 glutathione + 3 S-adenosyl-L-homocysteine + 3 H(+)

  Catalysed by GLYAT, GLYATL2, GLYATL1, GSS and 1 moremitochondrial matrixOne of 3 routes

• D-glucosyl-N-acylsphingosineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. sphing-4-enine + a fatty acyl-CoA = an N-acylsphing-4-enine + CoA + H(+)
  3. UDP-glucose:N-acylsphingosine D-glucosyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatus, cytosolOne of 2 routes

• a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. sphing-4-enine + a fatty acyl-CoA = an N-acylsphing-4-enine + CoA + H(+)
  3. an N-acylsphing-4-enine + UDP-alpha-D-galactose = a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatusOne of 2 routes