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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-08-12 21:31:07 UTC
Update Date2019-07-25 18:12:30 UTC
HMDB IDHMDB0003464
Secondary Accession Numbers
  • HMDB0005991
  • HMDB03464
  • HMDB05991
Metabolite Identification
Common Name4-Guanidinobutanoic acid
Description4-Guanidinobutanoate is a normal metabolite present in low concentrations. Patients with hyperargininemia have an arginase deficiency which leads to blockade of the urea cycle in the last step with several clinical symptoms. Owing to the arginase deficiency this patients accumulate arginine which leads eventually to epileptogenic guanidino compounds (PMID 7752905 ).
Structure
Data?1563860748
Synonyms
ValueSource
4-GuanidinobutanoateChEBI
4-Guanidinobutyric acidChEBI
gamma-GuanidinobutyrateChEBI
gamma-Guanidinobutyric acidChEBI
4-GuanidinobutyrateGenerator
g-GuanidinobutyrateGenerator
g-Guanidinobutyric acidGenerator
Γ-guanidinobutyrateGenerator
Γ-guanidinobutyric acidGenerator
4-(Carbamimidamido)butanoateHMDB
4-(Carbamimidamido)butanoic acidHMDB
gamma-Guanidinobutyrate, monosodium saltHMDB
4-Guanidinobutanoic acidGenerator
Chemical FormulaC5H11N3O2
Average Molecular Weight145.1597
Monoisotopic Molecular Weight145.085126611
IUPAC Name4-[(diaminomethylidene)amino]butanoic acid
Traditional Name4-[(diaminomethylidene)amino]butanoic acid
CAS Registry Number463-00-3
SMILES
NC(N)=NCCCC(O)=O
InChI Identifier
InChI=1S/C5H11N3O2/c6-5(7)8-3-1-2-4(9)10/h1-3H2,(H,9,10)(H4,6,7,8)
InChI KeyTUHVEAJXIMEOSA-UHFFFAOYSA-N
Chemical Taxonomy
Description belongs to the class of organic compounds known as gamma amino acids and derivatives. These are amino acids having a (-NH2) group attached to the gamma carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentGamma amino acids and derivatives
Alternative Parents
Substituents
  • Gamma amino acid or derivatives
  • Straight chain fatty acid
  • Fatty acid
  • Fatty acyl
  • Guanidine
  • Organic 1,3-dipolar compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carboximidamide
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Organic nitrogen compound
  • Carbonyl group
  • Hydrocarbon derivative
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

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 Solubility5.13 g/LALOGPS
logP-1.2ALOGPS
logP-2.7ChemAxon
logS-1.4ALOGPS
pKa (Strongest Acidic)4.24ChemAxon
pKa (Strongest Basic)12.07ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area101.7 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity35.88 m³·mol⁻¹ChemAxon
Polarizability14.71 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-006x-9000000000-33270c51eae32ab986ffJSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00dl-9300000000-6b386451847864ccb5a6JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0002-2900000000-fde97cd959d0a824fb1aJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-000i-9000000000-7d6981f3d74ac35fbae3JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-052r-9000000000-566db10c9a42f3463f73JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-0006-0900000000-9133b9248cec58ba4611JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-0udi-0900000000-839146a1223d81fcfe1eJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0ue9-7900000000-e5db002aeb9f842883d4JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-000x-9000000000-272ecb5c7a854deaabb6JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0006-9000000000-dcdfb92af31da28b06e6JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-0002-0900000000-57afcc249d5db66b3fafJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-000j-9700000000-20d2b0d6d24335aaf532JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-000i-9000000000-a4bb465c44540f709872JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-00kr-9000000000-2803a79b84b073387a24JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-0007-9000000000-05ad8804b2be6f14b017JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-0900000000-9133b9248cec58ba4611JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0udi-0900000000-839146a1223d81fcfe1eJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0ue9-7900000000-e5db002aeb9f842883d4JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-000x-9000000000-272ecb5c7a854deaabb6JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9000000000-dcdfb92af31da28b06e6JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-0udi-0900000000-7f59dfb80b806d284f40JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-002b-2900000000-11fd478790c658cfaec3JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0iki-9400000000-483819543e16e57ade03JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03dl-9000000000-103ac42d2587c5c731b9JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0udl-3900000000-13045d3e8ab033475c4fJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0pb9-9500000000-087a77898c3c7b51800eJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9000000000-bb81f668fad53eb8c20bJSpectraViewer | MoNA
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR13C NMR SpectrumNot AvailableJSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableJSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.034 (0.013-0.055) uMAdult (>18 years old)Not SpecifiedNormal details
BloodDetected and Quantified0.21 (0.0-0.43) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified<0.0248 uMAdult (>18 years old)Both
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified0.0125 (0 - 0.025) uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified1.3 +/- 1.05 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected but not Quantified Adult (>18 years old)BothNormal details
UrineDetected and Quantified0.17 (0.0-0.37) umol/mmol creatinineAdult (>18 years old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.05 (0.013-0.09) uMAdult (>18 years old)BothCirrhosis details
BloodDetected and Quantified0.230 +/- 0.110 uMAdult (>18 years old)Both
Uremia
details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified1.0 +/- 0.8 umol/mmol creatinineAdult (>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 ]
Uremia
  1. Vanholder R, De Smet R, Glorieux G, Argiles A, Baurmeister U, Brunet P, Clark W, Cohen G, De Deyn PP, Deppisch R, Descamps-Latscha B, Henle T, Jorres A, Lemke HD, Massy ZA, Passlick-Deetjen J, Rodriguez M, Stegmayr B, Stenvinkel P, Tetta C, Wanner C, Zidek W: Review on uremic toxins: classification, concentration, and interindividual variability. Kidney Int. 2003 May;63(5):1934-43. doi: 10.1046/j.1523-1755.2003.00924.x. [PubMed:12675874 ]
Colorectal cancer
  1. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  2. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB002246
KNApSAcK IDNot Available
Chemspider ID486
KEGG Compound IDC01035
BioCyc IDCPD-592
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID6938
PubChem Compound500
PDB IDNot Available
ChEBI ID15728
Food Biomarker OntologyNot Available
VMH IDNot Available
References
Synthesis ReferencePisano, John J.; Mitoma, Chozo; Udenfriend, Sidney. Biosynthesis of g-guanidinobutyric acid from g-aminobutyric acid and arginine. Nature (London, United Kingdom) (1957), 180 1125-6.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. De Deyn PP, Marescau B, Cuykens JJ, Van Gorp L, Lowenthal A, De Potter WP: Guanidino compounds in serum and cerebrospinal fluid of non-dialyzed patients with renal insufficiency. Clin Chim Acta. 1987 Jul 30;167(1):81-8. [PubMed:3665089 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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. [PubMed:16460903 ]
  6. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]

Enzymes

General function:
Amino acid transport and metabolism
Specific function:
Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis.
Gene Name:
GATM
Uniprot ID:
P50440
Molecular weight:
48455.01
Reactions
L-Arginine + gamma-Aminobutyric acid → Ornithine + 4-Guanidinobutanoic aciddetails