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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-08-13 14:46:20 UTC
Update Date2017-12-07 01:53:31 UTC
HMDB IDHMDB0004225
Secondary Accession Numbers
  • 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 ).
Structure
Thumb
Synonyms
ValueSource
2-oxo-5-guanidino-PentanoateChEBI
2-oxo-5-GuanidinopentanoateChEBI
2-oxo-5-Guanidinovaleric acidChEBI
2-OxoarginineChEBI
5-((Aminoiminomethyl)amino)-2-oxopentanoic acidChEBI
5-guanidino-2-oxo-PentanoateChEBI
5-guanidino-2-OxopentanoateChEBI
alpha-keto-delta-Guanidinopentanoic acidChEBI
2-oxo-5-guanidino-Pentanoic acidGenerator
a-keto-delta-GuanidinovalerateGenerator
a-keto-delta-Guanidinovaleric acidGenerator
alpha-keto-delta-GuanidinovalerateGenerator
α-keto-δ-guanidinovalerateGenerator
α-keto-δ-guanidinovaleric acidGenerator
2-oxo-5-Guanidinopentanoic acidGenerator
2-oxo-5-GuanidinovalerateGenerator
5-((Aminoiminomethyl)amino)-2-oxopentanoateGenerator
5-guanidino-2-oxo-Pentanoic acidGenerator
5-guanidino-2-Oxopentanoic acidGenerator
a-keto-delta-GuanidinopentanoateGenerator
a-keto-delta-Guanidinopentanoic acidGenerator
alpha-keto-delta-GuanidinopentanoateGenerator
α-keto-δ-guanidinopentanoateGenerator
α-keto-δ-guanidinopentanoic acidGenerator
a-keto-δ-guanidinovalerateGenerator
a-keto-δ-guanidinovaleric acidGenerator
a-keto-δ-guanidinopentanoateGenerator
a-keto-δ-guanidinopentanoic acidGenerator
5-(diaminomethylideneamino)-2-OxopentanoateHMDB
5-(diaminomethylideneamino)-2-Oxopentanoic acidHMDB
5-guanidino-2-Oxovaleric acidHMDB
a-keto-D-Guanidinovaleric acidHMDB
D-guanidino-a-Oxovaleric acidHMDB
D-guanido-a-Ketovaleric acidHMDB
delta-guanidino-alpha-Oxovaleric acidHMDB
delta-guanido-alpha-Ketovaleric acidHMDB
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
DescriptionThis compound belongs to the class of chemical entities known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassKeto 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

Health effect:

  Health condition:

    Endocrine disorders:

    Gastrointestinal disorders:

    Psychiatric disorders:

    Renal and urinary disorders:

    Nervous system disorders:

  Observation:

    Investigations:

Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

  Cell and elements:

Source:

Route of exposure:

  Parenteral:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

    Cellular process:

Role

Indirect biological role:

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility4.06 g/LALOGPS
logP-1.3ALOGPS
logP-2.4ChemAxon
logS-1.6ALOGPS
pKa (Strongest Acidic)3.01ChemAxon
pKa (Strongest Basic)10.71ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area118.77 ŲChemAxon
Rotatable Bond Count5ChemAxon
Refractivity41.23 m³·mol⁻¹ChemAxon
Polarizability16.72 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0096-9100000000-303513b0b209bca56206View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00r6-9710000000-95bf9765fd1fa4c2cd82View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-001i-0900000000-4c337e4db09d448ecc63View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0563-9600000000-d97800b4db5ae44aebbaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-004i-9200000000-6286bbf95e4a4716e7d4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0ab9-1900000000-05cd70e6e18e08b35be8View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0900-7900000000-aec8a82d0097632e2cc6View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03dl-9000000000-c27deb914dc894c12d6cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-001i-2900000000-67d1c875d2da6e075630View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-001i-9800000000-1a3c6500b4d652bcaadbView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9000000000-89a2ffbc759f6a2a5922View in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Peroxisome
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
Tissue LocationNot Available
Pathways
NameSMPDB/PathwhizKEGG
D-Arginine and D-Ornithine MetabolismPw000019Pw000019 greyscalePw000019 simpleMap00472
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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
Cerebrospinal Fluid (CSF)Detected and Quantified0.12 +/- 0.12 (0- 0.25) uMAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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
Associated Disorders and Diseases
Disease References
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 ]
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 ]
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 ]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB 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 ID58489
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 ]

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