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

1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
2. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

Catalysed by AGXT2, PIPOXOne of 2 routes

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

Catalysed by AGXT2, SHMT1, SHMT2

1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
2. L-Citrulline:L-aspartate ligase (AMP-forming)

Catalysed by DDAH1, ASS1cytosolOne of 6 routes

1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
2. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

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

1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
2. IMP:L-aspartate ligase (GDP-forming)

Catalysed by AGXT2, ADSS2, ADSS1cytosolOne of 2 routes

1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
2. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

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

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)

Catalysed by SLC25A2, SLC7A6inner side of the membrane, outer side of the membraneOne of 5 routes

1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-ornithine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-ornithine(in)
2. D-ornithine(in) + L-ornithine(out) = D-ornithine(out) + L-ornithine(in)

Catalysed by SLC25A2inner side of the membrane, outer side of the membraneOne of 2 routes

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

Catalysed by AGXT2, GLYATL3, GLYAT, GLYATL2

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-Arginine:glycine amidinotransferase

Catalysed by SLC25A2, GATMinner side of the membrane, outer side of the membrane, cytosolOne of 4 routes

1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
2. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
2. transport of citrulline and methionine (cytosol to extracellular)

Catalysed by DDAH1, SLC7A6cytosol, extracellular spaceOne of 23 routes

1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
2. transport of citrulline and tryptophan (cytosol to extracellular)

Catalysed by DDAH1, SLC7A9, SLC3A1, SLC7A6cytosol, extracellular spaceOne of 16 routes

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(+)

Catalysed by AGXT2, GSS

1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 4 routes

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(+)

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

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(+)

Catalysed by AGXT2, KYNU

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)

Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membraneOne of 4 routes

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. leukotriene C4 hydrolysis

Catalysed by AGXT2cytosol

1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
2. transport of asparagine and citrulline (cytosol to extracellular)

Catalysed by DDAH1, SLC7A9, SLC3A1, SLC7A6cytosol, extracellular spaceOne of 17 routes

1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)
2. taurine + L-arginine = taurocyamine + L-ornithine

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

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

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

1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
2. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

Catalysed by AGXT2, AGA

1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
2. transport of citrulline and phenylalanine (cytosol to extracellular)

Catalysed by DDAH1, SLC7A6cytosol, extracellular spaceOne of 23 routes

1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
2. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

Catalysed by AGXT2, KYNU

1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
2. Carbamoyl-phosphate:L-ornithine carbamoyltransferase

Catalysed by DDAH1, OTCcytosol, mitochondrial matrixOne of 2 routes

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

Catalysed by AGXT2, AADATOne of 3 routes

1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
2. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

Catalysed by AGXT2, SLC1A4inner side of the membrane, outer side of the membraneOne of 2 routes

1. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
2. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

Catalysed by AGXT2, PAICScytosolOne of 2 routes

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-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

Catalysed by SLC25A2, IL4I1inner side of the membrane, outer side of the membrane

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 + pyruvate = 5-amino-2-oxopentanoate + L-alanine

Catalysed by SLC25A2, AGXT2inner side of the membrane, outer side of the membraneOne of 2 routes

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

Catalysed by AGXT2, GLYATL2One of 2 routes

1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
2. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

Catalysed by AGXT2, SLC1A4inner side of the membrane, outer side of the membraneOne of 2 routes

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. Hippurate (Benzoylglycinate) Formation

Catalysed by AGXT2mitochondrial matrixOne of 2 routes

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)

Catalysed by AGXT2, SLC25A12, SLC25A13mitochondrial matrix, cytosolOne of 4 routes

1. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
2. gamma-glutamyltransferase (alanine)

Catalysed by AGXT2, GGTLC3, GGT1, GGT5 and 3 morecytosolOne of 2 routes

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-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

Catalysed by SLC25A2, NOS3, NOS1, NOS2inner side of the membrane, outer side of the membrane, cytosol

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

Catalysed by AGXT2, MOCOS

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. Glycodeoxycholate amidohydrolase

Catalysed by AGXT2cytosolOne of 2 routes

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

Catalysed by AGXT2, GLYATL2

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

Catalysed by AGXT2, BAAT

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

Catalysed by AGXT2, BAAT

1. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
2. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

Catalysed by AGXT2, BAAT

1. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase
2. transport of citrulline and tryptophan (cytosol to extracellular)
3. beta-carboline hydrolysis

Catalysed by DDAH1, SLC7A9, SLC3A1, SLC7A6cytosol, extracellular space

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. Carnitine Transport by Atb0