N(omega),N('omega)-dimethyl-L-arginine

Three steps

• Met-tRNA(Met)Reach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and methionine (cytosol to extracellular)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by AGXT2, SLC7A5, MARS1extracellular space, cytosol

• (S)-lactateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by AGXT2, LDHA, LDHB, LDHC and 2 more

• 3'-deoxy-3',4'-didehydro-CTPReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. CTP + AH2 + S-adenosyl-L-methionine = 3'-deoxy-3',4'-didehydro-CTP + 5'-deoxyadenosine + L-methionine + A + H2O + H(+)

  Catalysed by AGXT2, MOCOS, RSAD2

• pyridoxineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine-5'-phosphate phosphohydrolase

  Catalysed by AGXT2, SCLY, PHOSPHO2, PDXPcytosolOne of 2 routes

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by AGXT2, AADATOne of 2 routes

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by AGXT2, SLC7A5, CNDP2extracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by AGXT2One of 7 routes

• tryptamineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

  Catalysed by AGXT2, SLC7A5, DDCextracellular space, cytosol

• 2-oxoglutaramateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by AGXT2, KYAT1cytosolOne of 5 routes

• (S)-2-hydroxyglutarateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (S)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2

  Catalysed by AGXT2, MOCOS, L2HGDH

• formaldehydeReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. sarcosine:oxygen oxidoreductase (demethylating)

  Catalysed by AGXT2, GNMT, PIPOX, SARDHcytosol, peroxisomeOne of 2 routes

• 3-fumarylpyruvateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by AGXT2, FAHD1

• trans-1,2-dithiane-4,5-diolReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. selenocysteine lyase (dithiothreitol)

  Catalysed by AGXT2, SCLYcytosol

• creatineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. L-Arginine:glycine amidinotransferase
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by AGXT2, GATM, GAMTcytosolOne of 2 routes

• N-acetyl-L-aspartateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by AGXT2, NAT8Lmitochondrial matrixOne of 2 routes

• bilirubin IXalphaReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of bilirubin and GSH (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• oxalic acidReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and chloride (cytosol to extracellular)
  3. transport of chloride and oxalate (cytosol to extracellular)

  Catalysed by AGXT2, SLC6A14, SLC26A6extracellular space, cytosolOne of 3 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. transport of (R)-3-hydroxybutanoate and pyruvate (cytosol to extracellular)

  Catalysed by AGXT2, SLC16A4, SLC16A5, SLC16A3 and 3 moreextracellular space, cytosolOne of 2 routes

• taurolithocholateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and taurolithocholate (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• 3-hydroxypyruvateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by AGXT2, AGXTOne of 2 routes

• CHEBI:17208Reach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by AGXT2, GSScytosol

• homocysteineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Formation of Homocystine

  Catalysed by AGXT2, GSScytosol

• L-glutamic 5-semialdehydeReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and ornithine (cytosol to extracellular)
  3. L-Ornithine:2-oxo-acid aminotransferase

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

• anilineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by AGXT2, NAT2, NAT1mitochondrial matrix, cytosol

• aceneuramateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by AGXT2, NPL

• CHEBI:17312Reach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by AGXT2, TYW1B, TYW1One of 5 routes

• sulfiteReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  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 AGXT2, GSS, TSTD1One of 2 routes

• CHEBI:17380Reach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by AGXT2, SLC7A5extracellular space, cytosolOne of 3 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by AGXT2, SLC7A5, PRODH2extracellular space, cytosolOne of 3 routes

• agmatineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by AGXT2, SLC7A6extracellular space, cytosol, mitochondrial matrix

• indole-3-pyruvateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  3. L-tryptophan + glyoxylate = indole-3-pyruvate + glycine

  Catalysed by AGXT2, AADATOne of 3 routes

• 3,3',5'-triiodo-L-thyronine sulfateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3',5'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2

  Catalysed by AGXT2, MOCOS, DIO1

• 3,3'-diiodo-L-thyronineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3One of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2

  Catalysed by AGXT2, MOCOS, DIO1One of 2 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by AGXT2, HADHA, ACADVL, HADHBmitochondrial matrix

• CHEBI:17724Reach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by AGXT2, GNMT, PDPR, DMGDHcytosol

• glycine betaineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and chloride (cytosol to extracellular)
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by AGXT2, SLC6A14, SLC6A12extracellular space, cytosolOne of 3 routes

• trans-urocanateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by AGXT2, SLC7A6, HALextracellular space, cytosol

• urateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. urate(out) + glutathione(in) = urate(in) + glutathione(out)

  Catalysed by AGXT2, GSS, SLC22A13inner side of the membrane, outer side of the membrane

• 5-hydroxy-L-tryptophanReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine 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 AGXT2, SLC7A5, TPH1, TPH2extracellular space, cytosol

• methylarsonous acidReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by AGXT2, GSS, GSTO1, GSTO2

• 4-methyl-2-oxopentanoateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  3. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine

  Catalysed by AGXT2, AADATOne of 5 routes

• CHEBI:17927Reach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 2 routes

• 3-oxopropanoic acidReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by AGXT2, SLC7A10, ABATinner side of the membrane, outer side of the membrane, mitochondrial matrix

• 3-phenylpyruvateReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  3. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine

  Catalysed by AGXT2, AADATOne of 6 routes

• dTDPReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. pyruvate kinase (dTDP)

  Catalysed by AGXT2, PKM, PKLRcytosol

• CHEBI:18183Reach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. gamma-glutamyltransferase (alanine)
  3. gamma-glutamylcyclotransferase ((5-L-glutamyl)-L-amino acid)

  Catalysed by AGXT2, GGT1, GGT5, GGTLC2 and 5 moreextracellular space, cytosol

• N-carbamoyl-beta-alanineReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. transport of alanine and chloride (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by AGXT2, SLC6A14extracellular space, cytosolOne of 2 routes

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by AGXT2, GARTcytosol