1.0 2011-09-21 00:24:55 UTC 2015-10-09 22:32:39 UTC FDB023116 Guanidinosuccinic acid Guanidinosuccinic acid (GSA) is one of the earliest uremic toxins isolated and its toxicity identified. Its metabolic origins show that it arose from the oxidation of argininosuccinic acid (ASA) by free radicals. The stimulus for this oxidation, occurring optimally in the presence of the failed kidney, is the rising level of urea which, through enzyme inhibition, results in a decline in hepatic levels of the semi-essential amino acid, arginine. It is further noted that concentrations of GSA in both serum and urine decline sharply in animals and humans exposed to the essential amino acid, methionine. Uremic patients suffer from a defective ability to generate methyl groups due to anorexia, dietary restrictions and renal protein leakage. This leads to the accumulation of homocysteine, a substance known to produce vascular damage. Even in healthy subjects intake of choline together with methionine is insufficient to satisfy total metabolic requirements for methyl groups. In end-stage renal disease, therefore, protein restriction contributes to the build-up of toxins in uremia. Replacement using specific amino acid mixtures should be directed toward identified deficiencies and adequacy monitored by following serum levels of the related toxins, in this case GSA and homocysteine. (PMID 12701806) [HMDB] (2S)-2-Carbamimidamidobutanedioate (2S)-2-Carbamimidamidobutanedioic acid Guanidinosuccinate L-N-Amidinoaspartate L-N-amidinoaspartic acid N-(Aminoiminomethyl)-L-aspartate N-(aminoiminomethyl)-L-aspartic acid N-Amidino-L-aspartate N-amidino-L-aspartic acid N-carbamimidoyl-L-aspartic acid C5H9N3O4 175.1427 175.059305791 (2S)-2-carbamimidamidobutanedioic acid N-amidino-L-aspartic acid 6133-30-8 NC(=N)N[C@@H](CC(O)=O)C(O)=O InChI=1S/C5H9N3O4/c6-5(7)8-2(4(11)12)1-3(9)10/h2H,1H2,(H,9,10)(H,11,12)(H4,6,7,8)/t2-/m0/s1 VVHOUVWJCQOYGG-REOHCLBHSA-N belongs to the class of organic compounds known as aspartic acid and derivatives. Aspartic acid and derivatives are compounds containing an aspartic acid or a derivative thereof resulting from reaction of aspartic acid at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom. Aspartic acid and derivatives Organic compounds Organic acids and derivatives Carboxylic acids and derivatives Amino acids, peptides, and analogues Aliphatic acyclic compounds Carbonyl compounds Carboximidamides Carboxylic acids Dicarboxylic acids and derivatives Fatty acids and conjugates Guanidines Hydrocarbon derivatives Organic oxides Organopnictogen compounds Propargyl-type 1,3-dipolar organic compounds Aliphatic acyclic compound Aspartic acid or derivatives Carbonyl group Carboximidamide Carboxylic acid Dicarboxylic acid or derivatives Fatty acid Guanidine Hydrocarbon derivative Organic 1,3-dipolar compound Organic nitrogen compound Organic oxide Organic oxygen compound Organonitrogen compound Organooxygen compound Organopnictogen compound Propargyl-type 1,3-dipolar organic compound C4-dicarboxylic acid L-aspartic acid derivative N-amidino-amino acid guanidines Solid logp -1.55 ALOGPS logs -1.79 ALOGPS solubility 2.82e+00 g/l ALOGPS logp -3.3 ChemAxon pka_strongest_acidic 2.98 ChemAxon pka_strongest_basic 12.23 ChemAxon iupac (2S)-2-carbamimidamidobutanedioic acid ChemAxon average_mass 175.1427 ChemAxon mono_mass 175.059305791 ChemAxon smiles NC(=N)N[C@@H](CC(O)=O)C(O)=O ChemAxon formula C5H9N3O4 ChemAxon inchi InChI=1S/C5H9N3O4/c6-5(7)8-2(4(11)12)1-3(9)10/h2H,1H2,(H,9,10)(H,11,12)(H4,6,7,8)/t2-/m0/s1 ChemAxon inchikey VVHOUVWJCQOYGG-REOHCLBHSA-N ChemAxon polar_surface_area 136.5 ChemAxon refractivity 47.25 ChemAxon polarizability 15.13 ChemAxon rotatable_bond_count 4 ChemAxon acceptor_count 7 ChemAxon donor_count 5 ChemAxon physiological_charge -1 ChemAxon formal_charge 0 ChemAxon Specdb::MsIr 1889 Specdb::MsIr 1890 Specdb::MsIr 1891 Specdb::CMs 17590 Specdb::CMs 38535 Specdb::CMs 155085 Specdb::NmrTwoD 1868 Specdb::NmrOneD 1933 Specdb::NmrOneD 129378 Specdb::NmrOneD 129379 Specdb::NmrOneD 129380 Specdb::NmrOneD 129381 Specdb::NmrOneD 129382 Specdb::NmrOneD 129383 Specdb::NmrOneD 129384 Specdb::NmrOneD 129385 Specdb::NmrOneD 129386 Specdb::NmrOneD 129387 Specdb::NmrOneD 129388 Specdb::NmrOneD 129389 Specdb::NmrOneD 129390 Specdb::NmrOneD 129391 Specdb::NmrOneD 129392 Specdb::NmrOneD 129393 Specdb::NmrOneD 129394 Specdb::NmrOneD 129395 Specdb::NmrOneD 129396 Specdb::NmrOneD 129397 Specdb::NmrOneD 166597 Specdb::MsMs 2206 Specdb::MsMs 2207 Specdb::MsMs 2208 Specdb::MsMs 304927 Specdb::MsMs 304928 Specdb::MsMs 304929 Specdb::MsMs 348259 Specdb::MsMs 348260 Specdb::MsMs 348261 Specdb::MsMs 2226406 Specdb::MsMs 2226989 Specdb::MsMs 2227710 Specdb::MsMs 2228326 Specdb::MsMs 2228806 Specdb::MsMs 2229419 Specdb::MsMs 2230068 Specdb::MsMs 2230617 Specdb::MsMs 2231694 Specdb::MsMs 2232479 Specdb::MsMs 2233089 Specdb::MsMs 2234071 Specdb::MsMs 2234886 Specdb::MsMs 2259022 Specdb::MsMs 2259542 Specdb::MsMs 2699755 HMDB03157 17072 #<Reference:0x000055ce306a1810> #<Reference:0x000055ce306a15b8> #<Reference:0x000055ce306a1360> Anatidae Type 1 specific Anatidae 8830 Beefalo Type 1 specific Bos taurus X Bison bison 297284 Bison Type 1 specific Bison bison 9901 Buffalo Type 1 specific Bubalus bubalis 89462 Cattle (Beef, Veal) Type 1 specific Bos taurus 9913 Chicken Type 1 specific Gallus gallus 9031 Columbidae (Dove, Pigeon) Type 1 specific Columbidae 8930 Deer Type 1 specific Cervidae 9850 Domestic goat Type 1 specific Capra aegagrus hircus 9925 Domestic pig Type 1 specific Sus scrofa domestica 9825 Elk Type 1 specific Cervus canadensis 1574408 Emu Type 1 specific Dromaius novaehollandiae 8790 European rabbit Type 1 specific Oryctolagus 9984 Greylag goose Type 1 specific Anser anser 8843 Guinea hen Type 1 specific Numida meleagris 8996 Horse Type 1 specific Equus caballus 9796 Mallard duck Type 1 specific Anas platyrhynchos 8839 Mountain hare Type 1 specific Lepus timidus 62621 Mule deer Type 1 specific Odocoileus 9871 Ostrich Type 1 specific Struthio camelus 8801 Pheasant Type 1 specific Phasianus colchicus 9054 Quail Type 1 specific Phasianidae 9005 Rabbit Type 1 specific Leporidae 9979 Rock ptarmigan Type 1 specific Lagopus muta 64668 Sheep (Mutton, Lamb) Type 1 specific Ovis aries 9940 Squab Type 1 specific Columba 8931 Turkey Type 1 specific Meleagris gallopavo 9103 Velvet duck Type 1 specific Melanitta fusca 371864 Wild boar Type 1 specific Sus scrofa 9823