You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:03:10 UTC
Secondary Accession NumbersNone
Metabolite Identification
Common NameHomo-L-arginine
DescriptionHomoarginine is a guanidino compounds of guanidinoethanesulfonic acid. It is an organ-specific uncompetitive inhibitor of human liver and bone alkaline phosphohydrolase. (PMID 5063678 ).
Chemical FormulaC7H16N4O2
Average Molecular Weight188.2275
Monoisotopic Molecular Weight188.127325776
IUPAC Name(2S)-2-amino-6-carbamimidamidohexanoic acid
Traditional NameL-homoarginine
CAS Registry Number156-86-5
InChI Identifier
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
  • L-alpha-amino acid
  • Medium-chain fatty acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • Guanidine
  • Carboximidamide
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Imine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
StatusDetected and Quantified
  • Endogenous
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
Experimental Properties
Melting Point213 - 215 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility1.58 mg/mLALOGPS
pKa (Strongest Acidic)2.49ChemAxon
pKa (Strongest Basic)12.3ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count5ChemAxon
Polar Surface Area125.22 Å2ChemAxon
Rotatable Bond Count6ChemAxon
Refractivity58.52 m3·mol-1ChemAxon
Polarizability20.04 Å3ChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-1900000000-be624ee9b24e82eb0742View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-001i-9100000000-f4d4f5ac890116d181f0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-053r-9000000000-4414ba2f5a6f7d2b30ccView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-000i-0900000000-46385e830fe015de53abView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-0002-0900000000-c6d6b6435e521a93b453View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0002-0900000000-6e5c16a8db2bbd58844fView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0002-6900000000-a844ac9257f1ef5a3b23View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0006-9000000000-f496e2b96544aa9df21dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-000i-0900000000-860f0c1c052c981b2f3bView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-001c-1900000000-6ea950560557eb3afd98View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-001i-9300000000-d110eb7150669bcdc29cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-001i-9000000000-2f0ed2e02fdc77532125View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-001i-9000000000-d1b2d06e85c976b4efa5View in MoNA
1D NMR13C NMR SpectrumNot Available
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Intestine
  • Testes
PathwaysNot Available
Normal Concentrations
BloodDetected and Quantified2.00 +/- 0.60 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified6.0 (0.52-12.3) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.12 +/- 0.60 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.7 +/- 0.4 uMAdult (>18 years old)MaleNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.7 +/- 0.2 uMAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified0.04342 +/- 0.01184 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified0.98 (0.0-1.98) umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BloodDetected and Quantified1.65 +/- 0.70 uMAdult (>18 years old)BothCirrhosis details
Cerebrospinal Fluid (CSF)Detected and Quantified1.16 uMAdult (>18 years old)BothHyperargininemia details
Associated Disorders and Diseases
Disease References
  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. [7752905 ]
  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. [3433275 ]
Associated OMIM IDs
DrugBank IDDB03974
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB001490
KNApSAcK IDC00001364
Chemspider ID8732
KEGG Compound IDC01924
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00670
Metagene LinkHMDB00670
PubChem Compound9085
ChEBI ID27747
Synthesis ReferenceIzumiya, Nobuo. Synthesis of DL-homoarginine and Na-methyl-DL-homoarginine. Nippon Kagaku Zasshi (1951), 72 521-3. CODEN: NPKZAZ ISSN:0369-5387. CAN 46:42183 AN 1952:42183
Material Safety Data Sheet (MSDS)Download (PDF)
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. [3433275 ]
  2. Mori A, Watanabe Y, Fujimoto N: Fluorometrical analysis of guanidino compounds in human cerebrospinal fluid. J Neurochem. 1982 Feb;38(2):448-50. [7108550 ]
  3. Tamura Y, Fujii S: Purification of human plasma kallikrein and urokinase by affinity chromatography. J Biochem (Tokyo). 1976 Sep;80(3):507-11. [824284 ]
  4. Shoda J, Tanaka N, Osuga T, Matsuura K, Miyazaki H: Altered bile acid metabolism in liver disease: concurrent occurrence of C-1 and C-6 hydroxylated bile acid metabolites and their preferential excretion into urine. J Lipid Res. 1990 Feb;31(2):249-59. [2324645 ]
  5. Lin CW, Fishman WH: L-Homoarginine. An organ-specific, uncompetitive inhibitor of human liver and bone alkaline phosphohydrolases. J Biol Chem. 1972 May 25;247(10):3082-7. [5063678 ]
  6. 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. [3987036 ]
  7. Brock DJ: Amniotic fluid alkaline phosphatase isoenzymes in early prenatal diagnosis of cystic fibrosis. Lancet. 1983 Oct 22;2(8356):941-3. [6138505 ]
  8. Crofton PM, Smith AF: Alkaline phosphatase of high and low molecular mass in human serum and bile: a comparative study of kinetic properties. Clin Chem. 1980 Mar;26(3):451-6. [7363466 ]
  9. el-Mokhefi Z, Duvivier J, Plomteux G, Gielen J: [Biochemical characterization of a high-molecular weight alkaline phosphatase in a patient with cholangiocarcinoma (alpha-1 alkaline phosphatase)] Ann Biol Clin (Paris). 1985;43(3):215-20. [2411171 ]
  10. Muller F, Berg S, Frot JC, Boue J, Boue A: Prenatal diagnosis of cystic fibrosis. I. Prospective study of 51 pregnancies. Prenat Diagn. 1985 Mar-Apr;5(2):97-108. [2581242 ]
  11. Chang CH, Angellis D, Fishman WH: Presence of the rare D-variant heat-stable, placental-type alkaline phosphatase in normal human testis. Cancer Res. 1980 May;40(5):1506-10. [6154531 ]
  12. Gatti R, Gioia MG: Anisoin: a useful pre-chromatographic derivatization fluorogenic reagent for LC analysis of guanidino compounds. J Pharm Biomed Anal. 2006 Sep 11;42(1):11-6. Epub 2006 Feb 3. [16460903 ]
  13. 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. [1690873 ]
  14. Suzuki K, Yoshimura Y, Hisada Y, Matsumoto A: Sensitivity of intestinal alkaline phosphatase to L-homoarginine and its regulation by subunit-subunit interaction. Jpn J Pharmacol. 1994 Feb;64(2):97-102. [7518015 ]


General function:
Involved in oxidoreductase activity
Specific function:
Produces nitric oxide (NO) which is implicated in vascular smooth muscle relaxation through a cGMP-mediated signal transduction pathway. NO mediates vascular endothelial growth factor (VEGF)-induced angiogenesis in coronary vessels and promotes blood clotting through the activation of platelets. Isoform eNOS13C: Lacks eNOS activity, dominant-negative form that may down-regulate eNOS activity by forming heterodimers with isoform 1.
Gene Name:
Uniprot ID:
Molecular weight:
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [17016423 ]
  3. Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. [10592235 ]