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Record Information
StatusDetected and Quantified
Creation Date2006-05-22 15:12:37 UTC
Update Date2020-10-09 21:09:02 UTC
Secondary Accession Numbers
  • HMDB03157
Metabolite Identification
Common NameGuanidinosuccinic acid
DescriptionGuanidinosuccinic acid (GSA) has been identified as a uremic toxin according to the European Uremic Toxin Working Group (PMID: 22626821 ). It 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 ).
(2S)-2-Carbamimidamidobutanedioic acidChEBI
L-N-Amidinoaspartic acidChEBI
N-(Aminoiminomethyl)-L-aspartic acidChEBI
N-Amidino-L-aspartic acidKegg
Guanidinosuccinic acid, (DL)-isomerHMDB
N-Carbamimidoyl-L-aspartic acidHMDB
Guanidinosuccinic acidChEBI
Chemical FormulaC5H9N3O4
Average Molecular Weight175.1427
Monoisotopic Molecular Weight175.059305791
IUPAC Name(2S)-2-carbamimidamidobutanedioic acid
Traditional NameN-amidino-L-aspartic acid
CAS Registry Number6133-30-8
InChI Identifier
Chemical Taxonomy
Description 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.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentAspartic acid and derivatives
Alternative Parents
  • Aspartic acid or derivatives
  • Dicarboxylic acid or derivatives
  • Fatty acid
  • Guanidine
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carboximidamide
  • Carboxylic acid
  • Organopnictogen compound
  • Hydrocarbon derivative
  • Organic oxygen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic nitrogen compound
  • Carbonyl group
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Physiological effect

Health effect:


Route of exposure:


Biological location:

Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogP-2.068Not Available
Predicted Properties
Water Solubility2.82 g/LALOGPS
pKa (Strongest Acidic)2.98ChemAxon
pKa (Strongest Basic)12.23ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count7ChemAxon
Hydrogen Donor Count5ChemAxon
Polar Surface Area136.5 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity47.25 m³·mol⁻¹ChemAxon
Polarizability15.13 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-000x-9600000000-6898be602fa231388ab5Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-00di-9282000000-37ec05543d3d8d30ad15Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-004i-1900000000-466615ceb5129f085333Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-00di-9000000000-e5868bbf32c1407fa030Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-006x-9000000000-f34cd801b9c1e714a6fcSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0089-1900000000-ab305bd5f1306019d2bbSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0a5i-9500000000-e46a9bb7a8167700416dSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9000000000-cf381fa0309c6e1df17dSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0a4i-0900000000-766bed5b22ec642474b7Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-090r-6900000000-c59abb9d78dd85396ad9Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03di-9000000000-809ece24ff681a989230Spectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue LocationsNot Available
Normal Concentrations
BloodDetected and Quantified0.75 (0.37-1.13) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.259 +/- 0.096 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.4 +/- 0.2 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.17 +/- 0.057 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.4 +/- 0.2 uMAdult (>18 years old)Not SpecifiedNormal
    • Geigy Scientific ...
BloodDetected and Quantified0.171 +/- 0.0571 uMAdult (>18 years old)Both
BloodDetected and Quantified0.18 +/- 0.14 uMAdult (>18 years old)BothNormal details
Cellular CytoplasmDetected and Quantified0.2 +/- 0.5 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
Cerebrospinal Fluid (CSF)Detected and Quantified0.09 (0.05-0.13) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified<8.6 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
UrineDetected and Quantified4.0 +/- 2.0 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
    • West Cadwell, N.J...
    • Basel, Switzerlan...
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.48 (1.06-1.90) umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BloodDetected and Quantified0.11 +/- 0.106 uMAdult (>18 years old)BothCirrhosis details
BloodDetected and Quantified20.1 +/- 1.6 uMAdult (>18 years old)BothChronic renal failure details
BloodDetected and Quantified8.17 +/- 1.71 uMAdult (>18 years old)Bothuremia details
BloodDetected and Quantified37.143 +/- 19.429 uMAdult (>18 years old)Both
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
Associated Disorders and Diseases
Disease References
  1. Marescau B, De Deyn PP, Holvoet J, Possemiers I, Nagels G, Saxena V, Mahler C: Guanidino compounds in serum and urine of cirrhotic patients. Metabolism. 1995 May;44(5):584-8. [PubMed:7752905 ]
Chronic renal failure
  1. Kikuchi T, Orita Y, Ando A, Mikami H, Fujii M, Okada A, Abe H: Liquid-chromatographic determination of guanidino compounds in plasma and erythrocyte of normal persons and uremic patients. Clin Chem. 1981 Nov;27(11):1899-902. [PubMed:7296840 ]
  1. Duranton F, Cohen G, De Smet R, Rodriguez M, Jankowski J, Vanholder R, Argiles A: Normal and pathologic concentrations of uremic toxins. J Am Soc Nephrol. 2012 Jul;23(7):1258-70. doi: 10.1681/ASN.2011121175. Epub 2012 May 24. [PubMed:22626821 ]
  2. Vanholder R, De Smet R, Glorieux G, Argiles A, Baurmeister U, Brunet P, Clark W, Cohen G, De Deyn PP, Deppisch R, Descamps-Latscha B, Henle T, Jorres A, Lemke HD, Massy ZA, Passlick-Deetjen J, Rodriguez M, Stegmayr B, Stenvinkel P, Tetta C, Wanner C, Zidek W: Review on uremic toxins: classification, concentration, and interindividual variability. Kidney Int. 2003 May;63(5):1934-43. doi: 10.1046/j.1523-1755.2003.00924.x. [PubMed:12675874 ]
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB023116
KNApSAcK IDNot Available
Chemspider ID388951
KEGG Compound IDC03139
BioCyc IDCPD-599
BiGG IDNot Available
Wikipedia LinkNot Available
PubChem Compound439918
PDB IDNot Available
ChEBI ID17072
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00013446
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Torremans A, Marescau B, Van Dam D, Van Ginneken C, Van Meir F, Van Bogaert PP, D'Hooge R, de Vente J, De Deyn PP: GSA: behavioral, histological, electrophysiological and neurochemical effects. Physiol Behav. 2005 Feb 15;84(2):251-64. Epub 2005 Jan 8. [PubMed:15708777 ]
  2. Mizutani N, Hayakawa C, Ohya Y, Watanabe K, Watanabe Y, Mori A: Guanidino compounds in hyperargininemia. Tohoku J Exp Med. 1987 Nov;153(3):197-205. [PubMed:3433275 ]
  3. Cohen BD: Methyl group deficiency and guanidino production in uremia. Mol Cell Biochem. 2003 Feb;244(1-2):31-6. [PubMed:12701806 ]
  4. Duranton F, Cohen G, De Smet R, Rodriguez M, Jankowski J, Vanholder R, Argiles A: Normal and pathologic concentrations of uremic toxins. J Am Soc Nephrol. 2012 Jul;23(7):1258-70. doi: 10.1681/ASN.2011121175. Epub 2012 May 24. [PubMed:22626821 ]