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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2006-08-12 21:31:07 UTC
Update Date2020-10-09 21:03:10 UTC
HMDB IDHMDB0003464
Secondary Accession Numbers
  • HMDB0005991
  • HMDB03464
  • HMDB05991
Metabolite Identification
Common Name4-Guanidinobutanoic acid
Description4-Guanidinobutanoic acid, also known as gamma-guanidinobutyrate or 4-(carbamimidamido)butanoate, 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. 4-Guanidinobutanoic acid is a very strong basic compound (based on its pKa). 4-Guanidinobutanoic acid is a normal metabolite present in low concentrations. 4-Guanidinobutanoic acid exists in all eukaryotes, ranging from yeast to humans. Outside of the human body, 4-Guanidinobutanoic acid has been detected, but not quantified in, a few different foods, such as apples, french plantains, and loquats. This could make 4-guanidinobutanoic acid a potential biomarker for the consumption of these foods. 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?1582752278
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
4-[(Aminoiminomethyl)amino]butanoateHMDB
4-[(Aminoiminomethyl)amino]butanoic acidHMDB
N-(4-Butanoate)guanidineHMDB
N-(4-Butanoic acid)guanidineHMDB
gamma-GuanidinebutyrateHMDB
gamma-Guanidinebutyric acidHMDB
γ-GuanidinebutyrateHMDB
γ-Guanidinebutyric acidHMDB
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-1.5ALOGPS
logP-2.7ChemAxon
logS-1.7ALOGPS
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
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-006x-9000000000-33270c51eae32ab986ffSpectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00dl-9300000000-6b386451847864ccb5a6Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0002-2900000000-fde97cd959d0a824fb1aSpectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-000i-9000000000-7d6981f3d74ac35fbae3Spectrum
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-052r-9000000000-566db10c9a42f3463f73Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-0006-0900000000-9133b9248cec58ba4611Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-0udi-0900000000-839146a1223d81fcfe1eSpectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0ue9-7900000000-e5db002aeb9f842883d4Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-000x-9000000000-272ecb5c7a854deaabb6Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0006-9000000000-dcdfb92af31da28b06e6Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positivesplash10-0002-0900000000-57afcc249d5db66b3fafSpectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positivesplash10-000j-9700000000-20d2b0d6d24335aaf532Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positivesplash10-000i-9000000000-a4bb465c44540f709872Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positivesplash10-00kr-9000000000-2803a79b84b073387a24Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positivesplash10-0007-9000000000-05ad8804b2be6f14b017Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-0900000000-9133b9248cec58ba4611Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0udi-0900000000-839146a1223d81fcfe1eSpectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0ue9-7900000000-e5db002aeb9f842883d4Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-000x-9000000000-272ecb5c7a854deaabb6Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9000000000-dcdfb92af31da28b06e6Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-0udi-0900000000-7f59dfb80b806d284f40Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-002b-2900000000-11fd478790c658cfaec3Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0iki-9400000000-483819543e16e57ade03Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-03dl-9000000000-103ac42d2587c5c731b9Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0udl-3900000000-13045d3e8ab033475c4fSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0pb9-9500000000-087a77898c3c7b51800eSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0006-9000000000-bb81f668fad53eb8c20bSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR1H NMR SpectrumNot AvailableSpectrum
1D NMR13C NMR SpectrumNot AvailableSpectrum
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableSpectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Placenta
  • 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 QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified1.3 +/- 1.05 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>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 QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>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
FooDB IDFDB005408
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 ID4GUBUT
MarkerDB IDMDB00000424
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 ]
  7. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

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