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Human Metabolome Database Version 2.5

 

Showing metabocard for 2-Oxoarginine (HMDB04225)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2006-08-13 15:46:20
Update Date 2009-05-05 20:59:55
Accession Number HMDB04225
Secondary Accession Numbers HMDB06815
Common Name 2-Oxoarginine
Description 2-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)
Synonyms
  1. a-keto-d-Guanidinovaleric acid
  2. d-Guanidino-a-oxovaleric acid
  3. d-Guanido-a-ketovaleric acid
  4. 2-Oxo-5-guanidinovaleric acid
  5. 5-Guanidino-2-oxovaleric acid
  6. alpha-keto-delta-Guanidinovaleric acid
  7. delta-Guanidino-alpha-oxovaleric acid
  8. delta-Guanido-alpha-ketovaleric acid
  9. 5-(diaminomethylideneamino)-2-oxopentanoic acid
  10. 5-(diaminomethylideneamino)-2-oxopentanoate
Chemical IUPAC Name 5-(diaminomethylideneamino)-2-oxopentanoic acid
Chemical Formula C6H11N3O3
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Amino acids and Amino Acid conjugates
Class
  • Amino Acids
Sub Class
  • NA
Family
  • Mammalian Metabolite
Species
  • ketone
  • carboxylic acid
  • guanidine
Biofunction
  • Protein synthesis, amino acid biosynthesis
Application
—
Source
  • Endogenous
Average Molecular Weight 173.170
Monoisotopic Molecular Weight 173.080048
Isomeric SMILES NC(N)=NCCCC(=O)C(O)=O
Canonical SMILES NC(N)=NCCCC(=O)C(O)=O
KEGG Compound ID C05935 Link Image
BioCyc ID CPD-824 Link Image
BiGG ID 42500 Link Image
Wikipedia Link Not Available
NuGOwiki Link HMDB04225 Link Image
Metagene Link HMDB04225 Link Image
METLIN ID 7030 Link Image
PubChem Compound 558 Link Image
PubChem Substance 8222 Link Image
ChEBI ID Not Available
CAS Registry Number 3715-10-4
InChI Identifier InChI=1/C6H11N3O3/c7-6(8)9-3-1-2-4(10)5(11)12/h1-3H2,(H,11,12)(H4,7,8,9)
Synthesis Reference Chibata, Ichiro; Kakimoto, Toshio; Nabe, Koichi; Shibatani, Takeji. a-Keto-d-guanidinovalerianic acid. Jpn. Kokai Tokkyo Koho (1975), 4 pp.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 4.06 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge 0
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity -1.28 [Predicted by ALOGPS]; -1.3 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS) Not Available
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Not Available
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Not Available
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum
Low Energy
Download File
Show Experimental Conditions Link Image
Medium Energy
Download File
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High Energy
Download File
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Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm (Predicted from LogP)
  • Peroxisome
Biofluid Location
  • Blood
  • Cerebrospinal Fluid
Tissue Location Not Available
Concentrations (Normal)
Biofluid Blood
Value 0.145 +/- 0.007 uM
Age Adult:>18 yrs old
Sex Both
Patient information Euthyroidism
Comments Not Available
References
  • 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 Link Image]
Biofluid Blood
Value 0.10 (0.035-0.20) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid CSF
Value 0.12 +/- 0.12 (0- 0.25) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Concentrations (Abnormal)
Biofluid Blood
Value 0.134 +/- 0.014 uM
Age Adult:>18 yrs old
Sex Both
Condition Hypothyroidism
Comments Not Available
References
  • 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 Link Image]
Biofluid Blood
Value 0.160 +/- 0.009 uM
Age Adult:>18 yrs old
Sex Both
Condition Hyperthyroidism
Comments Not Available
References
  • 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 Link Image]
Biofluid Blood
Value 0.09 (0.035-0.150) uM
Age Adult:>18 yrs old
Sex Both
Condition Cirrhosis
Comments Not Available
References
  • 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 Link Image]
Associated Disorders
Condition References
Cirrhosis
  • 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 Link Image]
Hyperthyroidism
  • 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 Link Image]
Hypothyroidism
  • 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 Link Image]
OMIM ID
Pathways
Name SMPDB Link KEGG Link
D-Arginine and D-Ornithine Metabolism SMP00036 Link Image map00472 Link Image
General References
  1. 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 Link Image]
  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 Link Image]
  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 Link Image]
  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 Link Image]
  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 Link Image]
Metabolic Enzymes
  1. Amiloride-sensitive amine oxidase [copper-containing] precursor
Enzyme 1 [top]
Enzyme 1 ID 10601
Enzyme 1 Name Amiloride-sensitive amine oxidase [copper-containing] precursor
Enzyme 1 Synonyms
  1. Diamine oxidase
  2. DAO
  3. Amiloride-binding protein
  4. ABP
  5. Histaminase
  6. Kidney amine oxidase
  7. KAO
Enzyme 1 Gene Name ABP1
Enzyme 1 Protein Sequence >Amiloride-sensitive amine oxidase [copper-containing] precursor 
MPALGWAVAAILMLQTAMAEPSPGTLPRKAGVFSDLSNQELKAVHSFLWSKKELRLQPSS
TTTMAKNTVFLIEMLLPKKYHVLRFLDKGERHPVREARAVIFFGDQEHPNVTEFAVGPLP
GPCYMRALSPRPGYQSSWASRPISTAEYALLYHTLQEATKPLHQFFLNTTGFSFQDCHDR
CLAFTDVAPRGVASGQRRSWLIIQRYVEGYFLHPTGLELLVDHGSTDAGHWAVEQVWYNG
KFYGSPEELARKYADGEVDVVVLEDPLPGGKGHDSTEEPPLFSSHKPRGDFPSPIHVSGP
RLVQPHGPRFRLEGNAVLYGGWSFAFRLRSSSGLQVLNVHFGGERIAYEVSVQEAVALYG
GHTPAGMQTKYLDVGWGLGSVTHELAPGIDCPETATFLDTFHYYDADDPVHYPRALCLFE
MPTGVPLRRHFNSNFKGGFNFYAGLKGQVLVLRTTSTVYNYDYIWDFIFYPNGVMEAKMH
ATGYVHATFYTPEGLRHGTRLHTHLIGNIHTHLVHYRVDLDVAGTKNSFQTLQMKLENIT
NPWSPRHRVVQPTLEQTQYSWERQAAFRFKRKLPKYLLFTSPQENPWGHKRSYRLQIHSM
ADQVLPPGWQEEQAITWARYPLAVTKYRESELCSSSIYHQNDPWDPPVVFEQFLHNNENI
ENEDLVAWVTVGFLHIPHSEDIPNTATPGNSVGFLLRPFNFFPEDPSLASRDTVIVWPRD
NGPNYVQRWIPEDRDCSMPPPFSYNGTYRPV
Enzyme 1 Number of Residues 751
Enzyme 1 Molecular Weight 85342
Enzyme 1 Theoretical pI Not Available
Enzyme 1 GO Classification
Function
  • binding
  • cation binding
  • copper ion binding
  • ion binding
  • transition metal ion binding
Process
—
Component
—
Enzyme 1 General Function Secondary metabolites biosynthesis, transport and catabolism
Enzyme 1 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
Enzyme 1 Pathways
Enzyme 1 Reactions
  • H2O + Histamine + O2 --> Hydrogen peroxide + Imidazole-4-acetaldehyde + Ammonium
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • 1-19
Enzyme 1 Transmembrane Regions Not Available
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 177960 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID P19801 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name ABP1_HUMAN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >2142 bp 
ATGCCGGCCCTGGGCTGGGCCGTGGCTGCCATCCTGATGCTGCAGACGGCCATGGCGGAG
CCCTCCCCGGGGACTCTGCCCAGGAAGGCAGGGGTGTTTTCAGACCTAAGCAACCAAGAG
CTGAAGGCAGTGCACAGCTTCCTCTGGTCCAAGAAGGAGCTGAGGCTGCAGCCCTCCAGT
ACCACCACCATGGCCAAGAACACCGTGTTTCTCATCGAGATGCTGCTGCCCAAGAAGTAC
CATGTGCTGAGGTTTCTGGATAAAGGTGAAAGGCATCCTGTGCGGGAAGCCCGTGCCGTC
ATCTTCTTTGGTGACCAGGAGCATCCCAATGTCACCGAGTTTGCTGTGGGGCCCCTGCCA
GGGCCCTGCTACATGCGAGCACTGTCCCCCAGGCCTGGGTACCAGTCCTCCTGGGCATCG
AGGCCCATCTCCACAGCAGAGTATGCCCTCCTCTACCACACCCTGCAGGAAGCCACCAAG
CCCCTGCATCAGTTCTTCCTCAATACCACAGGCTTCTCATTCCAAGACTGCCATGACAGA
TGCCTGGCCTTCACCGATGTGGCCCCCCGGGGTGTGGCTTCTGGCCAGCGCCGCAGTTGG
CTTATCATACAGCGCTATGTAGAAGGCTACTTTCTGCACCCCACTGGGCTGGAGCTCCTC
GTGGATCATGGGAGCACAGATGCTGGGCACTGGGCCGTGGAGCAGGTGTGGTACAACGGG
AAGTTCTATGGGAGCCCAGAGGAACTGGCACGGAAGTATGCAGATGGAGAGGTGGACGTG
GTGGTCCTGGAGGACCGCTGCCTGGGGGCAAGGGGCATGACAGCACAGAGGAGCCGGCCC
TCTTCTCCTCCACAAGCCCCGCGGGACTTTCCCCAGCCCCATCCATGTGAGCGGCCCCCG
CTTGGTCCAGCCCCACGGCCCTCGCTTCAGGCTGGAGGGCAACGCTGTGCTCTACGGCGG
CTGGAGCTTTGCCTTCCGGTGCGCTCCTCCTCCGGGCTGCAGGTCCTGAACGTGCACTTC
GGCGGAGAGCGCATTGCCTATGAGGTCAGCGTGCAAGAGGCAGTGGCGCTGTATGGAGGA
CACACACCTGCAGGCATGCAGACCAAGTACCTCGATGTCGGCTGGGGCCTGGGCAGCGTC
ACTCATGAGTTAGCCCCCGGCATCGACTGCCCGGAGACCGCCACCTTCCTGGACACTTTC
CACTACTATGATGCCGATGACCCGGTCCATTATCCCCGAGCCCTCTGCCTCTTTGAAATG
CCCACAGGGGTGCCCCTTCGGCGGCACTTTAATTCCAACTTTAAAGGTGGCTTCAACTTC
TATGCAGGGCTGAAGGGCCAGGTGCTGGTGCTGCGGACAACTTCAACTGTCTACAATTAT
GATTACATTTGGGACTTTATCTTCTACCCCAACGGGGTGATGGAGGCCAAGATGCATGCC
ACTGGCTACGTCCACGCCACCTTCTACACCCCCGAGGGCTGCGCACGGCACTCGCCTGCA
CACCCACCTGATTGGCAACATACACACTCACTTGTGCACTACCGCGTAGACCTGGATGTG
GCAGGCACCAAGAACAGCTTCCAGACACTGCAGATGAAGCTAGAAAACATCACCAACCCC
TGGAGCCCGAGACACCGCGTGGTCCAGCCAACTCTGGAGCAGACGCAGTACTCCTGGGAG
CGCCAGGCGGCCTTCCGCTTCAAAAGGAGGCTGCCCAAGTACCTGCTCTTTACCAGCCCC
CAGGAGAACCCCTGGGGCCACAAGCGCAGCTACCGCCTGCAGATCCACTCCATGGCCGAC
CAGGTGCTGCCCCCAGGCTGGCAGGAGGAGCAGGCCATCACCTGGGCAAGGTACCCCCTG
GCAGTGACCAAGTACCGGGAGTCAGAGCTGTGCAGCAGCAGCATCTACCACCAGAACGAC
CCCTGGGACCCGCCCGTGGTCTTTGAGCAGTTTCTTCACAACAACGAGAACATTGAAAAT
GAGGACCTGGTGGCCTGGGTGACGGTGGGCTTCCTGCACATCCCCCACTCAGAGGACATT
CCCAACACAGCCACACCTGGGAACTCCGTGGGCTTCCTGCTCCGGCCATTCAACTTCTTC
CCAGAGGACCCCTCCCCTCCCTGGCATCCAGAGACACTGTGA
Enzyme 1 GenBank Gene ID M55602 Link Image
Enzyme 1 GeneCard ID ABP1 Link Image
Enzyme 1 GenAtlas ID ABP1 Link Image
Enzyme 1 HGNC ID HGNC:80 Link Image
Enzyme 1 Chromosome Location 7
Enzyme 1 Locus 7q34-q36
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. Barbry P, Champe M, Chassande O, Munemitsu S, Champigny G, Lingueglia E, Maes P, Frelin C, Tartar A, Ullrich A, et al.: Human kidney amiloride-binding protein: cDNA structure and functional expression. Proc Natl Acad Sci U S A. 1990 Oct;87(19):7347-51. [PubMed Link Image]
  2. Chassande O, Renard S, Barbry P, Lazdunski M: The human gene for diamine oxidase, an amiloride binding protein. Molecular cloning, sequencing, and characterization of the promoter. J Biol Chem. 1994 May 20;269(20):14484-9. [PubMed Link Image]
  3. Zhang X, Kim J, McIntire WS: cDNA sequences of variant forms of human placenta diamine oxidase. Biochem Genet. 1995 Aug;33(7-8):261-8. [PubMed Link Image]
  4. Novotny WF, Chassande O, Baker M, Lazdunski M, Barbry P: Diamine oxidase is the amiloride-binding protein and is inhibited by amiloride analogues. J Biol Chem. 1994 Apr 1;269(13):9921-5. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available