Record Information
Creation date2015-10-09 22:29:36 UTC
Update date2017-01-19 02:36:18 UTC
FoodComEx IDPC000278
FoodDB RecordFDB021885
Chemical Information
NameArgininosuccinic acid
DescriptionArginosuccinic 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. Defects in the arginosuccinate lyase enzyme can lead to arginosuccinate lyase deficiency. Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells and an excessive excretion of ASA in urine (arginosuccinic aciduria). In virtually all respects, this disorder shares the characteristics of other urea cycle defects. The most important characteristic of ASA lyase deficiency is its propensity to cause hyperammonemia in affected individuals. ASA in affected individuals is excreted by the kidney at a rate practically equivalent to the glomerular filtration rate (GFR). Whether ASA itself causes a degree of toxicity due to hepatocellular accumulation is unknown; such an effect could help explain hyperammonemia development in affected individuals. Regardless, the name of the disease is derived from the rapid clearance of ASA in urine, although elevated levels of ASA can be found in plasma. ASA lyase deficiency is associated with high mortality and morbidity rates. Symptoms of ASA lyase deficiency include anorexia, irritability rapid breathing, lethargy and vomiting. Extreme symptoms include coma and cerebral edema. [HMDB]
CAS Number2387-71-5
2-(N(omega)-L-arginine)succinic acidhmdb
2-(N(omega)-L-arginino)succinic acidhmdb
2-(Nomega-L-arginino)succinic acidGenerator
2-(Nw-L-arginino)butanedioic acidhmdb
Argininosuccinic acidhmdb
Arginosuccinic acidhmdb
L-Argininosuccinic acidhmdb
L-Arginosuccinic acidhmdb
N-(((4-amino-4-carboxybutyl)amino)iminomethyl)-L-Aspartic acidhmdb
N-(L-arginino) succinatehmdb
N-(L-arginino) succinic acidhmdb
N-(L-Arginino)succinic acidhmdb
N-[(4-amino-4-carboxybutyl)amidino]-L-Aspartic acidhmdb
N-[[(4-amino-4-carboxybutyl)amino]iminomethyl]-L-Aspartic acidhmdb
N(omega)-(L-arginino)succinic acidhmdb
Chemical FormulaC10H18N4O6
IUPAC name(2S)-2-[({2-[(4S)-4-amino-4-carboxybutyl]hydrazin-1-yl}methylidene)amino]butanedioic acid
InChI IdentifierInChI=1S/C10H18N4O6/c11-6(9(17)18)2-1-3-13-14-5-12-7(10(19)20)4-8(15)16/h5-7,13H,1-4,11H2,(H,12,14)(H,15,16)(H,17,18)(H,19,20)/t6-,7-/m0/s1
Isomeric SMILES[H][C@](N)(CCCNN=CN[C@@]([H])(CC(O)=O)C(O)=O)C(O)=O
Average Molecular Weight290.2731
Monoisotopic Molecular Weight290.122634328
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
  • L-alpha-amino acid
  • Alpha-amino acid
  • Tricarboxylic acid or derivatives
  • Amino acid
  • Carboxylic acid amidrazone
  • Formamidine
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carboxylic acid
  • Amidine
  • Organic nitrogen compound
  • Organic oxygen compound
  • Organopnictogen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Physico-Chemical Properties - Experimental
Experimental logPNot Available
Experimental Water SolubilityNot Available
Melting PointNot Available
Foods of Origin
FoodContent Range AverageReference
Production Data
Production Methodcommercial
Production Method ReferenceNot Available
Production Method Reference FileNot Available
Quantity AvailableProduction upon request, up to 5 mg
Delivery TimeNot Available
Storage Formsolid
Storage Conditions-80°C
StabilityNot Available
PurityNot Available
Spectral Data Upon RequestNot Available
Provider Information
Contact NameContact InstitutionContact Email
Rosa Vazquez
Commercial Vendors
Not Available