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

Three steps

• D-glucopyranose 6-phosphateReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  3. glucose-6-phosphatase (carbamoyl-phosphate)

  Catalysed by DDAH1, OTC, G6PC2cytosol, mitochondrial matrix, endoplasmic reticulum

• (R)-lactic 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. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by AGXT2, LDHDcytosol

• (S)-lactic 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. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by AGXT2, LDHB, LDHAL6Bmitochondrial matrixOne of 5 routes

• 25-hydroxycholesterolReach 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. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by AGXT2, MOCOS, CH25H

• 2-iminopropanoateReach 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-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by AGXT2, RIDA

• phosphoenolpyruvic 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. pyruvate kinase (dTDP)

  Catalysed by AGXT2, PKM, PKLRcytosolOne of 3 routes

• N-acetyl-beta-neuraminic 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. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by AGXT2, NPLcytosol

• tert-butanolReach 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. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by AGXT2, GSS, CLIC2, GPX1 and 3 more

• taurineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  2. transport of arginine and chloride (cytosol to extracellular)
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by SLC25A2, SLC6A14, SLC6A6inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 5 routes

• all-trans-13,14-dihydroretinolReach 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. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by AGXT2, MOCOS, RETSAT

• 3,3',5-triiodo-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',5-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO2One of 3 routes

• 3,3',5'-triiodo-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',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3

• a 3-acylpyruvateReach 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. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by AGXT2, FAHD1

• (R)-methylmalonyl-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. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by AGXT2, ALDH6A1mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-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. (S)-methylmalonyl-CoA hydrolase

  Catalysed by AGXT2mitochondrial matrix

• 3'-dephospho-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. acid phosphatase 2, lysosomal (CoA)

  Catalysed by AGXT2, ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-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. (2E)-dodecenoyl-CoA formation

  Catalysed by AGXT2mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-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. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by AGXT2, ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-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. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• benzoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by AGXT2, GLYATmitochondrial matrix

• butanoyl-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. Butanoate:CoA ligase (AMP-forming)

  Catalysed by AGXT2, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-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. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 10 routes

• glutaryl-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. Production of Glutaryl Carnitine

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• hexadecanoyl-CoAReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by AGXT2, GLYATL3

• eicosanoyl-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. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 12 routes

• itaconyl-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. Itaconate:CoA ligase (GDP-forming)

  Catalysed by AGXT2, SUCLG1, SUCLG2, SUCLA2mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-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. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 14 routes

• tetradecanoyl-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. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 12 routes

• octanoyl-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. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by AGXT2, CROTmitochondrial matrixOne of 21 routes

• phytanoyl-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. phytanate-CoA ligase

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 5 routes

• octadecanoyl-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. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 4 moremitochondrial matrix, cytosolOne of 15 routes

• prostaglandin E1Reach 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 prostaglandin E1 (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin I2Reach 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 prostaglandin I2 (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin F2alphaReach 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 prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin H2Reach 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. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O

  Catalysed by AGXT2, MOCOS, PTGS1, PTGS2One of 4 routes

• prostaglandin D2Reach 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 prostaglandin D2 (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach 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. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by AGXT2, MOCOS, TBXAS1, PTGS2 and 1 moreOne of 3 routes

• 7,8-dihydrofolateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,6,7,8-tetrahydrofolate:NAD+ oxidoreductase

  Catalysed by AGXT2, SHMT1, DHFR, DHFR2cytosolOne of 3 routes

• 5-formimidoyltetrahydrofolateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

  Catalysed by AGXT2, SHMT1, FTCDcytosolOne of 2 routes

• leukotriene A4Reach 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 leukotriene A4 (cytosol to extracellular)

  Catalysed by AGXT2, GSS, SLCO2A1cytosol, extracellular spaceOne of 3 routes

• (R)-S-lactoylglutathioneReach 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. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by AGXT2, GSS, GLO1, GLOD4cytosolOne of 3 routes

• carnosineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  2. L-histidine(out) + L-arginine(in) + Na(+)(out) = L-histidine(in) + L-arginine(out) + Na(+)(in)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by SLC25A2, SLC7A6, CARNS1inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-oxoadipateReach 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-2-aminoadipate + glyoxylate = 2-oxoadipate + glycine

  Catalysed by AGXT2, AADAT

• L-dopaReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and phenylalanine (cytosol to extracellular)
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

  Catalysed by DDAH1, SLC7A6, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 3 routes

• biotinReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-lysine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-lysine(in)
  2. biocytin + H2O = biotin + L-lysine
  3. Biotinidase (Biotin), Mitochondrial

  Catalysed by SLC25A2, BTDinner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 5 routes

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by DDAH1, SLC7A6, GOT2, GOT1cytosol, extracellular spaceOne of 5 routes

• S-formylglutathioneReach 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. alcohol dehydrogenase (formaldehyde)

  Catalysed by AGXT2, GSS, ADH1C, ADH1A and 4 morecytosolOne of 2 routes

• 2-methyl-3-oxopropanoateReach 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. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by AGXT2

• sphing-4-enineReach 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. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by AGXT2, ASAH1, ACER2, ACER1 and 1 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• spermidineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase

  Catalysed by SLC25A2, SLC3A2, SLC7A7, SRMinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 7 routes