CHEBI:15971

Three steps

• N-carbamoyl-L-aspartateReach from here →
  1. transport of citrulline and histidine (cytosol to extracellular)
  2. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  3. carbamoyl phosphate + L-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)

  Catalysed by SLC7A5, SLC7A6, OTC, CADextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• 3-oxopropanoateReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. Nalpha-(beta-alanyl)-L-histidine hydrolase
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by CARNS1, CNDP1, CNDP2, AGXT2cytosolOne of 2 routes

• thiosulfateReach from here →
  1. transport of cysteine and histidine (cytosol to extracellular)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate:sulfide sulfurtransferase

  Catalysed by SLC7A6, GOT2, GOT1, MPST and 1 moreextracellular space, cytosolOne of 6 routes

• tetra-mu3-sulfido-tetrairon(2+)Reach from here →
  1. transport of cysteine and histidine (cytosol to extracellular)
  2. De novo synthesis of 2Fe2S
  3. Synthesis of 4Fe4S from 2Fe2S

  Catalysed by SLC7A6, NDUFAB1, FDXR, FXN and 10 moreextracellular space, cytosol, mitochondrial matrix

• serotoninReach from here →
  1. L-histidine carboxy-lyase (histamine-forming)
  2. transport of histamine (cytosol to extracellular)
  3. transport of serotonin (cytosol to extracellular)

  Catalysed by HDC, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 8 routes

• an acylcholineReach from here →
  1. transport of chloride and histidine (cytosol to extracellular)
  2. transport of chloride and choline (cytosol to extracellular)
  3. an acylcholine + H2O = a carboxylate + choline + H(+)

  Catalysed by SLC6A14, SLC5A7, BCHEextracellular space, cytosol

• L-alpha-aminobutyric acidReach from here →
  1. transport of histidine and threonine (cytosol to extracellular)
  2. L-threonine ammonia-lyase (2-oxobutanoate-forming)
  3. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by SLC7A6, SDS, SDSL, GPTextracellular space, cytosol

• chenodeoxycholateReach from here →
  1. L-histidine carboxy-lyase (histamine-forming)
  2. transport of histamine (cytosol to extracellular)
  3. solute carrier organic anion transporter (chenodiol)

  Catalysed by HDC, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• taurocholateReach from here →
  1. L-histidine carboxy-lyase (histamine-forming)
  2. transport of histamine (cytosol to extracellular)
  3. solute carrier organic anion transporter (S-glutathionyl-2-4-dinitrobenzene)

  Catalysed by HDC, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 8 routes

• taurodeoxycholateReach from here →
  1. transport of chloride and histidine (cytosol to extracellular)
  2. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by SLC6A14, SLC6A6, BAATextracellular space, cytosolOne of 2 routes

• 3-hydroxyanthranilateReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by SLC7A6, KYNUextracellular space, cytosol

• N-formylanthranilic acidReach from here →
  1. transport of histidine and tryptophan (cytosol to extracellular)
  2. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)
  3. Formylkynurenine hydrolase

  Catalysed by SLC7A6, IDO1, TDO2, IDO2 and 1 moreextracellular space, cytosol

• 3-hydroxyisovaleric acidReach from here →
  1. transport of histidine and leucine (cytosol to extracellular)
  2. L-Leucine:2-oxoglutarate aminotransferase
  3. 4-methyl-2-oxopentanoate decarboxylation

  Catalysed by SLC7A6, BCAT2, ALOX12B, ALOXE3extracellular space, cytosol, mitochondrial matrix

• D-glucopyranose 6-phosphateReach from here →
  1. transport of citrulline and histidine (cytosol to extracellular)
  2. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  3. glucose-6-phosphatase (carbamoyl-phosphate)

  Catalysed by SLC7A5, SLC7A6, OTC, G6PC2extracellular space, cytosol, mitochondrial matrix, endoplasmic reticulum

• (R)-lactic acidReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by SLC7A6, AGXT2, LDHDextracellular space, cytosol, mitochondrial matrix

• (S)-lactic acidReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by SLC7A6, AGXT2, LDHB, LDHAL6Bextracellular space, cytosol, mitochondrial matrixOne of 7 routes

• 25-hydroxycholesterolReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by SLC7A6, MOCOS, CH25Hextracellular space, cytosol

• (6R)-L-erythro-6,7-dihydrobiopterinReach from here →
  1. transport of histidine and tryptophan (cytosol to extracellular)
  2. (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
  3. (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin = (6R)-L-erythro-6,7-dihydrobiopterin + H2O

  Catalysed by SLC7A6, TPH1, TPH2, PCBD1 and 1 moreextracellular space, cytosol

• 2-iminopropanoateReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by SLC7A6, AGXT2, RIDAextracellular space, cytosol, mitochondrial matrix

• phosphoenolpyruvic acidReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. pyruvate kinase (dTDP)

  Catalysed by SLC7A6, AGXT2, PKM, PKLRextracellular space, cytosol, mitochondrial matrix

• N-acetyl-beta-neuraminic acidReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by SLC7A6, AGXT2, NPLextracellular space, cytosol, mitochondrial matrix

• tert-butanolReach from here →
  1. transport of glycine and histidine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by SLC7A6, GSS, CLIC2, GPX1 and 3 moreextracellular space, cytosol

• 2-methyl-1-hydroxypropylthiamine diphosphateReach from here →
  1. transport of histidine and valine (cytosol to extracellular)
  2. L-Valine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)

  Catalysed by SLC7A6, BCAT2, BCKDHA, BCKDHBextracellular space, cytosol, mitochondrial matrix

• 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-oneReach from here →
  1. transport of histidine and methionine (cytosol to extracellular)
  2. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  3. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)

  Catalysed by SLC7A6, AADAT, ADI1extracellular space, cytosol

• Li(+)Reach from here →
  1. Formation of homocarnosine
  2. Transport of Homocarnosine
  3. transport of Li+ (cytosol to extracellular)

  Catalysed by CARNS1, SLC8B1cytosol, extracellular spaceOne of 3 routes

• all-trans-13,14-dihydroretinolReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by SLC7A6, MOCOS, RETSATextracellular space, cytosol

• 3,3',5-triiodo-L-thyronineReach from here →
  1. L-histidine carboxy-lyase (histamine-forming)
  2. transport of histamine (cytosol to extracellular)
  3. transport of S-glutathionyl-2-4-dinitrobenzene (cytosol to extracellular)

  Catalysed by HDC, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 10 routes

• 3,3',5'-triiodo-L-thyronineReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by SLC7A6, MOCOS, DIO1, DIO3extracellular space, cytosol

• a 3-acylpyruvateReach from here →
  1. transport of alanine and histidine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by SLC7A6, AGXT2, FAHD1extracellular space, cytosol, mitochondrial matrix

• (R)-methylmalonyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by CARNS1, NAA10, ALDH6A1cytosol, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by CARNS1, NAA10cytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by CARNS1, NAA10, ACP2cytosol, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by CARNS1, NAA10cytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by CARNS1, NAA10, ACAA1, MYO5Bcytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 7 routes

• benzoyl-CoAReach from here →
  1. transport of glycine and histidine (cytosol to extracellular)
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by SLC7A6, GLYATextracellular space, cytosol, mitochondrial matrix

• butanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by CARNS1, NAA10, ACSM4, ACSM1 and 4 morecytosolOne of 11 routes

• dodecanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 10 routes

• glutaryl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. Production of Glutaryl Carnitine

  Catalysed by CARNS1, NAA10, CPT1A, CPT1C and 1 morecytosolOne of 2 routes

• hexadecanoyl-CoAReach from here →
  1. transport of glycine and histidine (cytosol to extracellular)
  2. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by SLC7A6, GLYATL3extracellular space, cytosol

• eicosanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by CARNS1, NAA10, SUCLG1, SUCLG2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 14 routes

• tetradecanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 6 morecytosolOne of 12 routes

• octanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by CARNS1, NAA10, CROTcytosol, mitochondrial matrixOne of 21 routes

• phytanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. phytanate-CoA ligase

  Catalysed by CARNS1, NAA10cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. L-histidine:beta-alanine ligase (ADP-forming)
  2. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by CARNS1, NAA10, SLC27A2, ACSL4 and 5 morecytosolOne of 15 routes

• prostaglandin E1Reach from here →
  1. L-histidine carboxy-lyase (histamine-forming)
  2. transport of histamine (cytosol to extracellular)
  3. transport of prostaglandin E1 (cytosol to extracellular)

  Catalysed by HDC, SLCO1A2, SLCO1B3, SLCO1B1cytosol, extracellular spaceOne of 4 routes

• prostaglandin I2Reach from here →
  1. L-histidine carboxy-lyase (histamine-forming)
  2. transport of histamine (cytosol to extracellular)
  3. transport of prostaglandin I2 (cytosol to extracellular)

  Catalysed by HDC, SLCO1A2, SLCO1B3, SLCO1B1cytosol, extracellular spaceOne of 4 routes

• prostaglandin F2alphaReach from here →
  1. L-histidine carboxy-lyase (histamine-forming)
  2. transport of histamine (cytosol to extracellular)
  3. transport of prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by HDC, SLCO1A2, SLCO1B3, SLCO1B1cytosol, extracellular spaceOne of 4 routes