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

Three steps

• hypotaurineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. 3-sulfino-L-alanine + H(+) = hypotaurine + CO2

  Catalysed by AGXT2, SLC25A12, SLC25A13, GADL1 and 1 moremitochondrial matrix, cytosol

• N-sulfo-D-glucosamineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by AGXT2, SLC25A14, SLC25A30, SGSHinner side of the membrane, outer side of the membrane

• dehydroepiandrosterone 3-sulfateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by AGXT2, SLC25A14, SLC25A30, STSinner side of the membrane, outer side of the membrane

• L-dopaquinoneReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,L-dopa:oxygen oxidoreductase

  Catalysed by DDAH1, SLC7A6, TYRcytosol, extracellular spaceOne of 2 routes

• IMPReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. IMP:L-aspartate ligase (GDP-forming)
  3. IMP + L-aspartate + GTP = N(6)-(1,2-dicarboxyethyl)-AMP + GDP + phosphate + 2 H(+)

  Catalysed by AGXT2, ADSS2, ADSS1cytosol

• porphobilinogenReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
  3. 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing

  Catalysed by AGXT2, ALAS1, ALAS2, CPNE7 and 1 moremitochondrial matrix, cytosolOne of 2 routes

• L,L-cystathionineReach 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. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by AGXT2, NAGSmitochondrial matrix

• gamma-L-glutamyl-L-cysteineReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by DDAH1, SLC7A6, GCLC, GCLMcytosol, extracellular spaceOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by DDAH1, SLC7A5, SLC7A6cytosol, extracellular space, peroxisomeOne of 2 routes

• L-homocysteineReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by DDAH1, SLC7A6, BHMT2cytosol, extracellular space

• adenosine 5'-phosphosulfateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. ATP:sulfate adenylyltransferase

  Catalysed by AGXT2, SLC25A14, SLC25A30, PAPSS1 and 1 moreinner side of the membrane, outer side of the membrane, cytosolOne of 4 routes

• N,N-dimethylglycineReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2
  3. (6S)-5,6,7,8-tetrahydrofolyl-(gamma-L-Glu)(n) + N,N-dimethylglycine + oxidized [electron-transfer flavoprotein] + H(+) = (6R)-5,10-methylenetetrahydrofolyl-(gamma-L-Glu)(n) + sarcosine + reduced [electron-transfer flavoprotein]

  Catalysed by AGXT2, PIPOX, DMGDH

• S-methyl-L-methionineReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by DDAH1, SLC7A6, BHMT2cytosol, extracellular space

• N-acetylputrescineReach 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. acetyl-CoA:putrescine N-acetyltransferase

  Catalysed by SLC25A2, SLC3A2, SLC7A7, SAT1 and 1 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• 4-aminobutanalReach 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. putrescine + O2 + H2O = 4-aminobutanal + H2O2 + NH4(+)

  Catalysed by SLC25A2, SLC3A2, SLC7A7, AOC1inner side of the membrane, outer side of the membrane, extracellular space, cytosol

• L-glutamyl 5-phosphateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-ornithine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-ornithine(in)
  2. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate
  3. L-glutamate 5-semialdehyde + phosphate + NADP(+) = L-glutamyl 5-phosphate + NADPH + H(+)

  Catalysed by SLC25A2, OAT, ALDH18A1inner side of the membrane, outer side of the membrane

• glutathione disulfideReach 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-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  3. cystine to cysteine conversion

  Catalysed by SLC25A2, SLC7A9inner side of the membrane, outer side of the membrane, cytosolOne of 36 routes

• taurolithocholate 3-sulfateReach 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 sulfotaurolithocholate (cytosol to extracellular)

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

• (S)-2-amino-6-oxohexanoateReach 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. L-saccharopine + NADP(+) + H2O = L-lysine + 2-oxoglutarate + NADPH + H(+)
  3. L-saccharopine + NAD(+) + H2O = (S)-2-amino-6-oxohexanoate + L-glutamate + NADH + H(+)

  Catalysed by SLC25A2, AASSinner side of the membrane, outer side of the membrane

• N(1)-acetylspermidineReach 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. N(1)-acetylspermidine + O2 + H2O = 3-acetamidopropanal + putrescine + H2O2

  Catalysed by SLC25A2, SLC3A2, SLC7A7, PAOXinner side of the membrane, outer side of the membrane, extracellular space, cytosol

• an acyl-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. acyl-CoA:sphingosine N-acyltransferase

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

• 5-oxo-L-prolineReach 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. epsilon-(gamma-L-glutamyl)-L-lysine = 5-oxo-L-proline + L-lysine
  3. epsilon-(gamma-L-glutamyl)-L-lysine = 5-oxo-L-proline + L-lysine

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

• histamineReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and histidine (cytosol to extracellular)
  3. L-histidine carboxy-lyase (histamine-forming)

  Catalysed by DDAH1, SLC7A5, SLC7A6, HDCcytosol, extracellular spaceOne of 4 routes

• L-thyroxineReach 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 thyroxine (cytosol to extracellular)

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

• kynurenateReach 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-kynurenine + glyoxylate = kynurenate + glycine + H2O

  Catalysed by AGXT2, KYAT3, AADATOne of 17 routes

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach 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. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by AGXT2, SHMT1, ATICcytosolOne of 3 routes

• (3Z)-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. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by AGXT2, HADHA, ACAA2, HADHBmitochondrial matrixOne of 3 routes

• S-sulfanyl-L-cysteineReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

  Catalysed by DDAH1, SLC7A6, CARS2cytosol, extracellular space

• N-formyl-L-kynurenineReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by DDAH1, SLC7A9, SLC3A1, SLC7A6 and 3 morecytosol, extracellular spaceOne of 2 routes

• (3S)-citramalyl-CoAReach 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. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by AGXT2, CLYBL

• L-2-aminoadipateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. 2-oxoadipate + L-kynurenine = L-2-aminoadipate + kynurenate + H2O

  Catalysed by AGXT2, KYNU, AADAT

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-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. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by AGXT2, SLC27A2, SLC27A5mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and glutamine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by DDAH1, SLC7A6, PPATcytosol, extracellular spaceOne of 2 routes

• phosphoenolpyruvateReach 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 + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by AGXT2, PCK1, PCK2

• D-glucosamine 6-phosphateReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and glutamine (cytosol to extracellular)
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

  Catalysed by DDAH1, SLC7A6, GFPT2, GFPT1cytosol, extracellular space

• L-methionine (S)-S-oxideReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by DDAH1, SLC7A6, MSRAcytosol, extracellular space

• L-methionine (R)-S-oxideReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by DDAH1, SLC7A6, MSRB3, MSRB1 and 1 morecytosol, extracellular space

• deoxycholoyl-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. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by AGXT2, SLC27A2, SLC27A5mitochondrial matrix, cytosolOne of 4 routes

• S-sulfanylglutathioneReach 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. Sulfide:glutathione,quinone oxidoreductase

  Catalysed by AGXT2, GSS, SQORcytosol, mitochondrial matrixOne of 5 routes

• methylamineReach 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. N(omega)-methyl-L-arginine(in) + L-arginine(out) = N(omega)-methyl-L-arginine(out) + L-arginine(in)
  3. N(omega)-methyl-L-arginine + H2O = L-citrulline + methylamine

  Catalysed by SLC25A2, SLC25A29, DDAH1inner side of the membrane, outer side of the membrane

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by DDAH1, SLC7A6, PPCScytosol, extracellular spaceOne of 4 routes

• monodehydro-L-ascorbate radicalReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)
  3. N-(9Z-octadecenoyl)glycine + 2 L-ascorbate + O2 = N-(9Z-octadecenoyl)-(2S)-hydroxyglycine + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by AGXT2, GLYATL3, GLYATL2, PAMOne of 5 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine

  Catalysed by AGXT2, AADAT, PAH

• pyridoxal 5'-phosphateReach 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 + O2 = pyridoxal 5'-phosphate + H2O2

  Catalysed by AGXT2, SCLY, PNPOcytosol

• an L-alpha-amino acidReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. an L-alpha-amino acid(in) + L-kynurenine(out) = an L-alpha-amino acid(out) + L-kynurenine(in)

  Catalysed by AGXT2, KYNU, SLC7A11inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-aminobutanoateReach 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. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by SLC25A2, SLC6A14, SLC6A12inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 5 routes

• 3-iodo-L-tyrosineReach from here →
  1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
  2. transport of citrulline and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by DDAH1, SLC7A6, TPOcytosol, extracellular spaceOne of 2 routes

• dopamineReach 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 dopamine and GSH (cytosol to extracellular)

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

• glycolithocholate 3-sulfateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  2. bile acid-CoA:amino acid N-acyltransferase (glycine)
  3. sulfotransferase (glycolithocholate)

  Catalysed by AGXT2, BAAT, SULT2A1cytosolOne of 3 routes

• estrone 3-sulfateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by AGXT2, SLC25A14, SLC25A30, STSinner side of the membrane, outer side of the membrane