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Human Metabolome Database Version 3.5

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Showing metabocard for Homocitrulline (HMDB00679)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-05-29 13:28:26 -0600
HMDB ID HMDB00679
Secondary Accession Numbers None
Metabolite Identification
Common Name Homocitrulline
Description Homocitrulline is a metabolite that can be detected in larger amounts in the urine of individuals with urea cycle disorders (OMIM 238970 Link_out). The accumulation of carbamylphosphate due to depleted supply of ornithine for the urea cycle may be responsible for the enhanced synthesis of homocitrulline and homoarginine in some cases (PMID 2474087 Link_out).
Structure Thumb
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. Homo-L-citrulline
  2. Homocitrulline
  3. L-Homocitrulline
  4. N-e-Carbamyl-L-lysine
  5. N-epsilon-Carbamyl-L-lysine
  6. N6-carbamoyl-L-Lysine
  7. N6-carbamoyl-Lysine
Chemical Formula C7H15N3O3
Average Molecular Weight 189.2123
Monoisotopic Molecular Weight 189.111341361
IUPAC Name (2S)-2-amino-6-(carbamoylamino)hexanoic acid
Traditional IUPAC Name L-homocitrulline
CAS Registry Number 1190-49-4
SMILES N[C@@H](CCCCNC(N)=O)C(O)=O
InChI Identifier InChI=1S/C7H15N3O3/c8-5(6(11)12)3-1-2-4-10-7(9)13/h5H,1-4,8H2,(H,11,12)(H3,9,10,13)/t5-/m0/s1
InChI Key XIGSAGMEBXLVJJ-YFKPBYRVSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Amino Acids, Peptides, and Analogues
Class Amino Acids and Derivatives
Sub Class Alpha Amino Acids and Derivatives
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Amino Fatty Acids
  • Organic Compounds
  • Straight Chain Fatty Acids
  • amino acid zwitterion(ChEBI)
Substituents
  • Carboxylic Acid
  • Polyamine
  • Primary Aliphatic Amine (Alkylamine)
  • Urea
Direct Parent Alpha Amino Acids and Derivatives
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction
  • Protein synthesis, amino acid biosynthesis
Application Not Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 211 - 212 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 600 mg/mL Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 12.1 g/L ALOGPS
LogP -3.13 ALOGPS
LogP -3.5 ChemAxon
LogS -1.19 ALOGPS
pKa (strongest acidic) 2.35 ChemAxon
pKa (strongest basic) 9.53 ChemAxon
Hydrogen Acceptor Count 4 ChemAxon
Hydrogen Donor Count 4 ChemAxon
Polar Surface Area 118.44 A2 ChemAxon
Rotatable Bond Count 6 ChemAxon
Refractivity 45.93 ChemAxon
Polarizability 19.49 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
Spectra
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Urine
Tissue Location Not Available
Pathways Not Available
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified
5.0 (0.0-12.0) uM Newborn (0-30 days old) Both Normal
  • Geigy Scient...
Urine Detected and Quantified
6.0 (0-8.8) umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
7.5 (3.8-13.8) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
  • Bouatra, S. ...
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease References None
Associated OMIM IDs None
DrugBank ID Not Available
DrugBank Metabolite ID Not Available
Phenol Explorer Compound ID Not Available
Phenol Explorer Metabolite ID Not Available
FoodDB ID FDB022178
KNApSAcK ID Not Available
Chemspider ID 58582 Link_out
KEGG Compound ID C02427 Link_out
BioCyc ID CPD-161 Link_out
BiGG ID Not Available
Wikipedia Link Not Available
NuGOwiki Link HMDB00679 Link_out
Metagene Link HMDB00679 Link_out
METLIN ID 46 Link_out
PubChem Compound 65072 Link_out
PDB ID Not Available
ChEBI ID 17443 Link_out
References
Synthesis Reference Stark, George R. Reactions of cyanate with functional groups of proteins. III. Reactions with amino and carboxyl groups. Biochemistry (1965), 4(6), 1030-6.
Material Safety Data Sheet (MSDS) Download (PDF)
General References
  1. Kato T, Sano M, Mizutani N: Inhibitory effect of intravenous lysine infusion on urea cycle metabolism. Eur J Pediatr. 1987 Jan;146(1):56-8. Pubmed: 3107993 Link_out
  2. Tuchman M, Knopman DS, Shih VE: Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. Arch Neurol. 1990 Oct;47(10):1134-7. Pubmed: 2222247 Link_out
  3. Rajantie J, Simell O, Perheentupa J: Oral administration of epsilon N-acetyllysine and homocitrulline in lysinuric protein intolerance. J Pediatr. 1983 Mar;102(3):388-90. Pubmed: 6402575 Link_out
  4. Dionisi Vici C, Bachmann C, Gambarara M, Colombo JP, Sabetta G: Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: low creatine excretion and effect of citrulline, arginine, or ornithine supplement. Pediatr Res. 1987 Sep;22(3):364-7. Pubmed: 3116497 Link_out
  5. Hommes FA, Roesel RA, Metoki K, Hartlage PL, Dyken PR: Studies on a case of HHH-syndrome (hyperammonemia, hyperornithinemia, homocitrullinuria). Neuropediatrics. 1986 Feb;17(1):48-52. Pubmed: 3960284 Link_out
  6. Koshiishi I, Kobori Y, Imanari T: Determination of citrulline and homocitrulline by high-performance liquid chromatography with post-column derivatization. J Chromatogr. 1990 Oct 26;532(1):37-43. Pubmed: 2079537 Link_out
  7. Evered DF, Vadgama JV: Absorption of homocitrulline from the gastrointestinal tract. Br J Nutr. 1983 Jan;49(1):35-42. Pubmed: 6821687 Link_out
  8. Kato T, Sano M, Mizutani N, Hayakawa C: Homocitrullinuria and homoargininuria in hyperargininaemia. J Inherit Metab Dis. 1988;11(3):261-5. Pubmed: 2466169 Link_out
  9. Kato T, Sano M: Effect of ammonium chloride on homocitrulline and homoarginine synthesis from lysine. J Inherit Metab Dis. 1993;16(5):906-7. Pubmed: 7507534 Link_out
  10. Kato T, Sano M, Mizutani N: Homocitrullinuria and homoargininuria in lysinuric protein intolerance. J Inherit Metab Dis. 1989;12(2):157-61. Pubmed: 2474087 Link_out
  11. Zammarchi E, Donati MA, Filippi L, Resti M: Cryptogenic hepatitis masking the diagnosis of ornithine transcarbamylase deficiency. J Pediatr Gastroenterol Nutr. 1996 May;22(4):380-3. Pubmed: 8732901 Link_out
  12. Simell O, Mackenzie S, Clow CL, Scriver CR: Ornithine loading did not prevent induced hyperammonemia in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome. Pediatr Res. 1985 Dec;19(12):1283-7. Pubmed: 4080446 Link_out
  13. Kraus LM, Gaber L, Handorf CR, Marti HP, Kraus AP Jr: Carbamoylation of glomerular and tubular proteins in patients with kidney failure: a potential mechanism of ongoing renal damage. Swiss Med Wkly. 2001 Mar 24;131(11-12):139-4. Pubmed: 11416886 Link_out
  14. Kraus LM, Elberger AJ, Handorf CR, Pabst MJ, Kraus AP Jr: Urea-derived cyanate forms epsilon-amino-carbamoyl-lysine (homocitrulline) in leukocyte proteins in patients with end-stage renal disease on peritoneal dialysis. J Lab Clin Med. 1994 Jun;123(6):882-91. Pubmed: 8201267 Link_out