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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2006-08-12 21:31:07 UTC
Update Date2023-02-21 17:16:42 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 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
Thumb
Synonyms
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
Not AvailableNot Available
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M+H]+Baker132.830932474
[M+H]+Not Available130.5http://allccs.zhulab.cn/database/detail?ID=AllCCS00000502
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Placenta
  • Prostate
Pathways
Normal Concentrations
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
Good Scents 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)Not Available
General References
  1. 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 ]
  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:3433275 ]
  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. 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 ]
  5. 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 ]
  6. 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 ]
  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