Record Information
Version2.0
Creation Date2012-05-31 13:03:22 -0600
Update Date2015-09-13 12:56:09 -0600
Secondary Accession Numbers
  • ECMDB01006
Identification
Name:Argininosuccinic acid
Description:Arginosuccinic acid is a basic amino acid. Some cells synthesize it from citrulline, aspartic acid and use it as a precursor for arginine in the urea cycle or citrulline-NO cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase. Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase.
Structure
Thumb
Synonyms:
  • 2-(N(Omega)-L-arginine)succinate
  • 2-(N(Omega)-L-arginine)succinic acid
  • 2-(N(Omega)-L-arginino)succinate
  • 2-(N(Omega)-L-arginino)succinic acid
  • 2-(Nw-L-arginino)butanedioate
  • 2-(Nw-L-arginino)butanedioic acid
  • Argininosuccinate
  • Argininosuccinic acid
  • Arginosuccinate
  • Arginosuccinic acid
  • ArgSuccinate
  • ArgSuccinic acid
  • ASA
  • L-Argininosuccinate
  • L-Argininosuccinic acid
  • L-Arginosuccinate
  • L-Arginosuccinic acid
  • N(Omega)-(L-arginino)succinate
  • N(Omega)-(L-arginino)succinic acid
  • N-(((4-Amino-4-carboxybutyl)amino)iminomethyl)-L-Aspartate
  • N-(((4-Amino-4-carboxybutyl)amino)iminomethyl)-L-Aspartic acid
  • N-(L-Arginino) succinate
  • N-(L-Arginino) succinic acid
  • N-(L-Arginino)succinate
  • N-(L-Arginino)succinic acid
  • N-[(4-Amino-4-carboxybutyl)amidino]-L-Aspartate
  • N-[(4-Amino-4-carboxybutyl)amidino]-L-Aspartic acid
  • N-[[(4-Amino-4-carboxybutyl)amino]iminomethyl]-L-Aspartate
  • N-[[(4-Amino-4-carboxybutyl)amino]iminomethyl]-L-Aspartic acid
Chemical Formula:C10H18N4O6
Weight:Average: 290.2731
Monoisotopic: 290.122634328
InChI Key:KDZOASGQNOPSCU-WDSKDSINSA-N
InChI:InChI=1S/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)/t5-,6-/m0/s1
CAS number:2387-71-5
IUPAC Name:(2S)-2-{3-[(4S)-4-amino-4-carboxybutyl]carbamimidamido}butanedioic acid
Traditional IUPAC Name:argininosuccinic acid
SMILES:N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=O
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as aspartic acid and derivatives. These 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.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassCarboxylic acids and derivatives
Direct ParentAspartic acid and derivatives
Alternative Parents
Substituents
  • Aspartic acid or derivatives
  • Alpha-amino acid
  • L-alpha-amino acid
  • Tricarboxylic acid or derivatives
  • Guanidine
  • Amino acid
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carboximidamide
  • Carboxylic acid
  • Hydrocarbon derivative
  • Organopnictogen compound
  • Organic oxygen compound
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Organic nitrogen compound
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Physical Properties
State:Solid
Charge:-1
Melting point:Not Available
Experimental Properties:
PropertyValueSource
Predicted Properties
PropertyValueSource
Water Solubility0.456 mg/mLALOGPS
logP-3.2ALOGPS
logP-5.8ChemAxon
logS-2.8ALOGPS
pKa (Strongest Acidic)2.14ChemAxon
pKa (Strongest Basic)12.39ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count10ChemAxon
Hydrogen Donor Count7ChemAxon
Polar Surface Area185.83 Å2ChemAxon
Rotatable Bond Count9ChemAxon
Refractivity75.31 m3·mol-1ChemAxon
Polarizability27.6 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Biological Properties
Cellular Locations:Cytoplasm
Reactions:
SMPDB Pathways:
Aspartate metabolismPW000787 Pw000787Pw000787 greyscalePw000787 simple
arginine metabolismPW000790 Pw000790Pw000790 greyscalePw000790 simple
KEGG Pathways:
EcoCyc Pathways:Not Available
Concentrations
Not Available
Spectra
Spectra:
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-0007-0921100000-638b5887a29712423afdView in MoNA
GC-MSGC-MS Spectrum - GC-MSsplash10-0007-8790000000-bc8aa39ecc71a1589298View in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-0006-4101900000-c606d87ec7ffb8ef2cfaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-000i-0090000000-27e7b2225bca7ed0bfefView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-00di-0390000000-b66871354ad66743ff5fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-001i-1910000000-777b6f2263b573f38bcaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-001i-3900000000-26d6466903550c40fde6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-001r-8900000000-4e5455a5af85574ac9dfView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-0006-0090000000-79b7ca9043632dc877e1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-0006-0590000000-8fb4f4625f241d387e92View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-06di-3910000000-55530a4477a3fde8c1c1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-00di-9200000000-413c3029964c0386047aView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-00di-9000000000-6ffc3b2b5da5f69de80bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-060r-0950000000-129ee81e1370707d975dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-0a4i-0910000000-62431d9967ced6c96b28View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-03di-1980000000-27b5f442d411115ebaabView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-0a4i-1900000000-b57bcbf0c07e921767afView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-0006-3900000000-1fce5114c61349fc9065View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-014i-0900000000-25c094a4a18cfe463b6bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-00di-9200000000-e518e242f12e95bbddf9View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positivesplash10-00di-9000000000-932dfd009a8e5c41ec38View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-000i-0090000000-27e7b2225bca7ed0bfefView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00dj-0190000000-8ba86e35661de03d1049View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0092-2890000000-966d24fb4f820888318eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-004i-4900000000-5200a801423dd1a4b7a6View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-00ds-0290000000-a3397e8c303fc7e6bd83View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00e9-0940000000-a964172e915808d27899View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0ac3-8900000000-d4ac87f770202453bb7aView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
References
References:
  • Au WL, Lim TC, Seow DC, Koh PL, Loh NK, Lim MS, Tan IK, Yee WC: Serial diffusion-weighted magnetic resonance imaging in adult-onset citrullinaemia. J Neurol Sci. 2003 May 15;209(1-2):101-4. Pubmed: 12686410
  • Cohen BD: Methyl group deficiency and guanidino production in uremia. Mol Cell Biochem. 2003 Feb;244(1-2):31-6. Pubmed: 12701806
  • Fleisher LD, Rassin DK, Desnick RJ, Salwen HR, Rogers P, Bean M, Gaull GE: Argininosuccinic aciduria: prenatal studies in a family at risk. Am J Hum Genet. 1979 Jul;31(4):439-45. Pubmed: 484552
  • Glick NR, Snodgrass PJ, Schafer IA: Neonatal argininosuccinic aciduria with normal brain and kidney but absent liver argininosuccinate lyase activity. Am J Hum Genet. 1976 Jan;28(01):22-30. Pubmed: 174426
  • Kanehisa, M., Goto, S., Sato, Y., Furumichi, M., Tanabe, M. (2012). "KEGG for integration and interpretation of large-scale molecular data sets." Nucleic Acids Res 40:D109-D114. Pubmed: 22080510
  • Kleijer WJ, Garritsen VH, Linnebank M, Mooyer P, Huijmans JG, Mustonen A, Simola KO, Arslan-Kirchner M, Battini R, Briones P, Cardo E, Mandel H, Tschiedel E, Wanders RJ, Koch HG: Clinical, enzymatic, and molecular genetic characterization of a biochemical variant type of argininosuccinic aciduria: prenatal and postnatal diagnosis in five unrelated families. J Inherit Metab Dis. 2002 Sep;25(5):399-410. Pubmed: 12408190
  • Michels VV, Beaudet AL: Arginase deficiency in multiple tissues in argininemia. Clin Genet. 1978 Jan;13(1):61-7. Pubmed: 624188
  • Pelli N, Fensom AH, Slade C, Boa F, Mieli-Vergani G, Vergani D: Argininosuccinate lyase: a new autoantigen in liver disease. Clin Exp Immunol. 1998 Dec;114(3):455-61. Pubmed: 9844057
  • Reid Sutton V, Pan Y, Davis EC, Craigen WJ: A mouse model of argininosuccinic aciduria: biochemical characterization. Mol Genet Metab. 2003 Jan;78(1):11-6. Pubmed: 12559843
  • Scaglia F, Brunetti-Pierri N, Kleppe S, Marini J, Carter S, Garlick P, Jahoor F, O'Brien W, Lee B: Clinical consequences of urea cycle enzyme deficiencies and potential links to arginine and nitric oxide metabolism. J Nutr. 2004 Oct;134(10 Suppl):2775S-2782S; discussion 2796S-2797S. Pubmed: 15465784
  • Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. Pubmed: 19212411
  • Wasant P, Srisomsap C, Liammongkolkul S, Svasti J: Urea cycle disorders in Thai infants: a report of 5 cases. J Med Assoc Thai. 2002 Aug;85 Suppl 2:S720-31. Pubmed: 12403252
  • Winder, C. L., Dunn, W. B., Schuler, S., Broadhurst, D., Jarvis, R., Stephens, G. M., Goodacre, R. (2008). "Global metabolic profiling of Escherichia coli cultures: an evaluation of methods for quenching and extraction of intracellular metabolites." Anal Chem 80:2939-2948. Pubmed: 18331064
Synthesis Reference:Hagino, Koji; Nakanishi, Toshihide. Fermentative production of L-arginosuccinic acid. Jpn. Kokai Tokkyo Koho (1980), 3 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
External Links:
ResourceLink
CHEBI ID15682
HMDB IDHMDB00052
Pubchem Compound ID439998
Kegg IDC03406
ChemSpider ID17215918
WikipediaArgininosuccinic acid
BioCyc IDNot Available

Enzymes

General function:
Involved in argininosuccinate synthase activity
Specific function:
ATP + L-citrulline + L-aspartate = AMP + diphosphate + N(omega)-(L-arginino)succinate
Gene Name:
argG
Uniprot ID:
P0A6E4
Molecular weight:
49898
Reactions
ATP + L-citrulline + L-aspartate = AMP + diphosphate + N(omega)-(L-arginino)succinate.
General function:
Involved in argininosuccinate lyase activity
Specific function:
2-(N(omega)-L-arginino)succinate = fumarate + L-arginine
Gene Name:
argH
Uniprot ID:
P11447
Molecular weight:
50318
Reactions
2-(N(omega)-L-arginino)succinate = fumarate + L-arginine.