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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2006-08-13 14:46:20 UTC
Update Date2023-02-21 17:16:58 UTC
HMDB IDHMDB0004225
Secondary Accession Numbers
  • HMDB0006815
  • HMDB04225
  • HMDB06815
Metabolite Identification
Common Name2-Oxoarginine
Description2-Oxoarginine is a guanidino compound metabolite of arginine catabolism. 2-Oxoarginine levels are increased in patients with argininemia (OMIM:207800 ). Argininemia, characterized by arginase deficiency (EC 3.5.3.1, catalyzes the last step of the urea cycle) is an autosomal recessive inborn error of metabolism caused by a defect in the final step in the urea cycle, the hydrolysis of arginine to urea and ornithine. Accumulation of arginine metabolites (such as guanidino compounds) especially 2-oxoarginine, may produce the central nervous system damage in argininemia. (PMID: 3433275 , 1588833 , 1690873 , 819629 ). 2-Oxoarginine has also been identified as a uremic toxin according to the European Uremic Toxin Working Group (PMID: 22626821 ).
Structure
Thumb
Synonyms
Chemical FormulaC6H11N3O3
Average Molecular Weight173.1698
Monoisotopic Molecular Weight173.080041233
IUPAC Name5-[(diaminomethylidene)amino]-2-oxopentanoic acid
Traditional Name5-[(diaminomethylidene)amino]-2-oxopentanoic acid
CAS Registry Number3715-10-4
SMILES
NC(=N)NCCCC(=O)C(O)=O
InChI Identifier
InChI=1S/C6H11N3O3/c7-6(8)9-3-1-2-4(10)5(11)12/h1-3H2,(H,11,12)(H4,7,8,9)
InChI KeyARBHXJXXVVHMET-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassShort-chain keto acids and derivatives
Direct ParentShort-chain keto acids and derivatives
Alternative Parents
Substituents
  • Short-chain keto acid
  • Alpha-keto acid
  • Alpha-hydroxy ketone
  • Guanidine
  • Ketone
  • Carboxylic acid derivative
  • Carboxylic acid
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carboximidamide
  • Monocarboxylic acid or derivatives
  • Organic nitrogen compound
  • Organonitrogen compound
  • Organooxygen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Carbonyl group
  • Organic oxygen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Disposition
Biological locationRoute of exposureSource
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Peroxisome
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.145 +/- 0.007 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified0.10 (0.035-0.20) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified<0.200 uMAdult (>18 years old)Both
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.12 +/- 0.12 (0- 0.25) uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.23 (0.18-0.35) uMAdult (>18 years old)BothUremia details
BloodDetected and Quantified0.134 +/- 0.014 uMAdult (>18 years old)BothHypothyroidism details
BloodDetected and Quantified0.160 +/- 0.009 uMAdult (>18 years old)BothHyperthyroidism details
BloodDetected and Quantified0.09 (0.035-0.150) uMAdult (>18 years old)BothCirrhosis details
UrineDetected but not QuantifiedNot QuantifiedNot SpecifiedNot SpecifiedCancer patients undergoing total body irradiation details
Associated Disorders and Diseases
Disease References
Hypothyroidism
  1. Verhelst J, Berwaerts J, Marescau B, Abs R, Neels H, Mahler C, De Deyn PP: Serum creatine, creatinine, and other guanidino compounds in patients with thyroid dysfunction. Metabolism. 1997 Sep;46(9):1063-7. [PubMed:9284897 ]
Hyperthyroidism
  1. Verhelst J, Berwaerts J, Marescau B, Abs R, Neels H, Mahler C, De Deyn PP: Serum creatine, creatinine, and other guanidino compounds in patients with thyroid dysfunction. Metabolism. 1997 Sep;46(9):1063-7. [PubMed:9284897 ]
Cirrhosis
  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 ]
Uremia
  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 ]
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB023338
KNApSAcK IDNot Available
Chemspider ID542
KEGG Compound IDC03771
BioCyc IDCPD-824
BiGG ID42500
Wikipedia LinkNot Available
METLIN ID7030
PubChem Compound558
PDB IDNot Available
ChEBI ID28116
Food Biomarker OntologyNot Available
VMH ID5G2OXPT
MarkerDB IDMDB00000444
Good Scents IDNot Available
References
Synthesis ReferenceChibata, Ichiro; Kakimoto, Toshio; Nabe, Koichi; Shibatani, Takeji. a-Keto-d-guanidinovalerianic acid. Jpn. Kokai Tokkyo Koho (1975), 4 pp.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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 ]
  2. Marescau B, Qureshi IA, De Deyn P, Letarte J, Ryba R, Lowenthal A: Guanidino compounds in plasma, urine and cerebrospinal fluid of hyperargininemic patients during therapy. Clin Chim Acta. 1985 Feb 28;146(1):21-7. [PubMed:3987036 ]
  3. Marescau B, De Deyn PP, Lowenthal A, Qureshi IA, Antonozzi I, Bachmann C, Cederbaum SD, Cerone R, Chamoles N, Colombo JP, et al.: Guanidino compound analysis as a complementary diagnostic parameter for hyperargininemia: follow-up of guanidino compound levels during therapy. Pediatr Res. 1990 Mar;27(3):297-303. [PubMed:1690873 ]
  4. Marescau B, Deshmukh DR, Kockx M, Possemiers I, Qureshi IA, Wiechert P, De Deyn PP: Guanidino compounds in serum, urine, liver, kidney, and brain of man and some ureotelic animals. Metabolism. 1992 May;41(5):526-32. [PubMed:1588833 ]
  5. Wiechert P, Mortelmans J, Lavinha F, Clara R, Terheggen HG, Lowenthal A: Excretion of guanidino-derivates in urine of hyperargininemic patients. J Genet Hum. 1976 Mar;24(1):61-72. [PubMed:819629 ]
  6. 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 ]

Enzymes

General function:
Involved in copper ion binding
Specific function:
Catalyzes the degradation of compounds such as putrescine, histamine, spermine, and spermidine, substances involved in allergic and immune responses, cell proliferation, tissue differentiation, tumor formation, and possibly apoptosis. Placental DAO is thought to play a role in the regulation of the female reproductive function.
Gene Name:
ABP1
Uniprot ID:
P19801
Molecular weight:
85377.1