2-(N(omega)-L-arginino)succinate

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. transport of arginine and methionine (cytosol to extracellular)

Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 9 routes

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. transport of arginine and tryptophan (cytosol to extracellular)

Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 5 routes

1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
2. L-aspartate 1-carboxy-lyase (beta-alanine-forming)

Catalysed by ASS1, GAD2cytosolOne of 2 routes

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. transport of alanine and arginine (cytosol to extracellular)

Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 6 routes

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. transport of arginine and asparagine (cytosol to extracellular)

Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 5 routes

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. taurine + L-arginine = taurocyamine + L-ornithine

Catalysed by ASL, GATMcytosol

1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
2. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

Catalysed by ASS1, AGA

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. transport of arginine and phenylalanine (cytosol to extracellular)

Catalysed by ASL, SLC7A6cytosol, extracellular spaceOne of 8 routes

1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
2. Carbamoyl-phosphate:L-ornithine carbamoyltransferase

Catalysed by ASS1, OTCmitochondrial matrixOne of 2 routes

1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)

Catalysed by ASS1, SLC7A13inner side of the membrane, outer side of the membraneOne of 2 routes

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-arginine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-arginine(in)

Catalysed by ASL, SLC25A2cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase

Catalysed by ASL, ADSLcytosolOne of 4 routes

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

Catalysed by ASL, IL4I1cytosol

1. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)

Catalysed by ASS1, SLC25A12, SLC25A13mitochondrial matrix, cytosolOne of 4 routes

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

Catalysed by ASL, NOS3, NOS1, NOS2cytosol

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. transport of arginine and tryptophan (cytosol to extracellular)
3. beta-carboline hydrolysis

Catalysed by ASL, SLC7A6cytosol, extracellular space

1. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
2. transport of arginine and chloride (cytosol to extracellular)
3. Carnitine Transport by Atb0

Catalysed by ASL, SLC6A14