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
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-02-26 21:22:04 UTC
HMDB IDHMDB0000337
Secondary Accession Numbers
  • HMDB0006118
  • HMDB00337
  • HMDB06118
Metabolite Identification
Common Name(S)-3,4-Dihydroxybutyric acid
Description3,4-Dihydroxybutyric acid (also known as 3,4-Dihydroxybutanoic acid or 3,4-DB, or 2-deoxytetronic acid, or 2-DTA) is a normal human urinary metabolite that is excreted in increased concentration in patients with succinic semialdehyde dehydrogenase (SSADH) deficiency (PMID 12127325 ), which is a genetic disorder. SSADH deficiency is caused by an enzyme deficiency in GABA degradation. The most constant features with this disease are developmental delay, hypotonia and intellectual disability. Nearly half of SSADH patients exhibit ataxia, behavioral problems, seizures, and hyporeflexia. Normal adults excrete 0.37 +/- 0.15 (SD) mmoles of 3,4-dihydroxybutyrate per 24 hr. 3,4-dihydroxybutyrate is also detectable in blood. The renal clearance of 3,4-dihydroxybutyrate is roughly equal that of creatinine. 3,4-dihydroxybutyric acid has also been found in foods and is believed to be formed via the degradation (cooking) of di- and poly-saccharides (PMID: 1167165 ). 3,4-Dihydroxybutyric acid is believed to play a role in satiety or the feeling of fullness and suppresses food intake (PMID: 3191387 ; PMID: 6720928 ). 3,4-dihydroxybutyric acid is also elevated in the serum of individuals with dementia and may even serve as a predictive biomarker for dementia (PMID: 25177334 ). 3,4-Dihydroxybutyric acid is also a metabolite produced in Escherichia (PMID: 28342964 ).
Structure
Data?1582752124
Synonyms
ValueSource
(S)-3,4-DihydroxybutyrateGenerator
(S)-3,4-Dihydroxy-butyric acidHMDB
(S)-3,4-DihydroxybutanoateHMDB
(S)-3,4-Dihydroxybutanoic acidHMDB
2-DeoxytetronateHMDB
2-Deoxytetronic acidHMDB
3,4-DihydroxybutanoateHMDB
3,4-Dihydroxybutanoic acidHMDB
3,4-Dihydroxybutanoic acid, (S)-isomerHMDB
3,4-Dihydroxybutanoic acid, monosodium saltHMDB
Chemical FormulaC4H8O4
Average Molecular Weight120.1039
Monoisotopic Molecular Weight120.042258744
IUPAC Name3,4-dihydroxybutanoic acid
Traditional Name3,4-dihydroxybutanoic acid
CAS Registry Number51267-44-8
SMILES
OCC(O)CC(O)=O
InChI Identifier
InChI=1S/C4H8O4/c5-2-3(6)1-4(7)8/h3,5-6H,1-2H2,(H,7,8)
InChI KeyDZAIOXUZHHTJKN-UHFFFAOYSA-N
Chemical Taxonomy
Description belongs to the class of organic compounds known as beta hydroxy acids and derivatives. Beta hydroxy acids and derivatives are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassBeta hydroxy acids and derivatives
Direct ParentBeta hydroxy acids and derivatives
Alternative Parents
Substituents
  • Beta-hydroxy acid
  • Short-chain hydroxy acid
  • Fatty acid
  • 1,2-diol
  • Secondary alcohol
  • Carboxylic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Organic oxygen compound
  • Organooxygen compound
  • Primary alcohol
  • Hydrocarbon derivative
  • Carbonyl group
  • Organic oxide
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Biological location:

Source:

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 Solubility832 g/LALOGPS
logP-1.5ALOGPS
logP-1.4ChemAxon
logS0.84ALOGPS
pKa (Strongest Acidic)4.09ChemAxon
pKa (Strongest Basic)-2.9ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area77.76 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity25.01 m³·mol⁻¹ChemAxon
Polarizability10.91 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0012-0910000000-4054231c9c4cffa8a3c9Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0012-0910000000-4054231c9c4cffa8a3c9Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-08iu-9000000000-92e0a2008c94b2db1bc7Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positivesplash10-00di-9282000000-4da1d5189d3237b2686bSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0udr-5900000000-87e855dfa4a5229ec106Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0gw0-9300000000-88020d54d9b37645b873Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4u-9000000000-d0c64e75e2e9de34f683Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-7900000000-6aec80cde1bf2e0bca19Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0pxr-9300000000-93d09801aa0c902a4e8fSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9000000000-d765ea0631d195240881Spectrum
Biological Properties
Cellular LocationsNot Available
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified18.0 (0.0-54.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified15.0 +/- 15.0 uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Both
Normal
details
UrineDetected and Quantified67.400 +/- 56.200 umol/mmol creatinineInfant (0-1 year old)Both
Normal
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified21.2 (9.3-47.0) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified23.9 (12.4-31.8) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified10.2 (6.4-16.7) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified8.3 (4.8-22.4) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified73.0 +/- 24.0 umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified8.0 +/- 3.0 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified34.9 (12.5 - 57.2) umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified42.2 (21.9-56.1) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified137.5 (50.0-225.0) umol/mmol creatinineChildren (1-13 years old)Both4-Hydroxybutyric Aciduria
    • MetaGene: Metabol...
details
UrineDetected and Quantified810-1160 umol/mmol creatinineInfant (0-1 year old)Male
Succinic semialdehyde dehydrogenase deficiency
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
Associated Disorders and Diseases
Disease References
Succinic semialdehyde dehydrogenase deficiency
  1. Shinka T, Inoue Y, Ohse M, Ito A, Ohfu M, Hirose S, Kuhara T: Rapid and sensitive detection of urinary 4-hydroxybutyric acid and its related compounds by gas chromatography-mass spectrometry in a patient with succinic semialdehyde dehydrogenase deficiency. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Aug 25;776(1):57-63. [PubMed:12127325 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Associated OMIM IDs
  • 271980 (Succinic semialdehyde dehydrogenase deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB021961
KNApSAcK IDNot Available
Chemspider ID133026
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound150929
PDB IDNot Available
ChEBI ID86371
Food Biomarker OntologyNot Available
VMH IDNot Available
References
Synthesis ReferenceCho, Yik-haeng; Chun, Jongpil; Park, Youngmi; Roh, Kyoungrok; Yu, Hosung; Hwang, Daeil. Process for preparing optically pure (S)-3,4-dihydroxybutyric acid derivatives. PCT Int. Appl. (2000), 29 pp.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed:8087979 ]
  2. Ohdoi C, Nyhan WL, Kuhara T: Chemical diagnosis of Lesch-Nyhan syndrome using gas chromatography-mass spectrometry detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):123-30. [PubMed:12829005 ]
  3. Nakajima MO, Etoh Y, Yanai M, Kawamura R, Kaneko K, Aoyagi K, Koiso K: [Novel renal function marker, ATP--establishing the normal range, cases of anti-tumor drags administration for urinary-track tumor, diabetic diseases and a newborn baby]. Rinsho Byori. 2002 May;50(5):513-8. [PubMed:12078051 ]
  4. Fell V, Lee CR, Pollitt RJ: The occurrence of (S)-3,4-dihydroxybutyrate in human blood and urine. Biochem Med. 1975 May;13(1):40-5. [PubMed:1167165 ]
  5. Shinka T, Inoue Y, Ohse M, Ito A, Ohfu M, Hirose S, Kuhara T: Rapid and sensitive detection of urinary 4-hydroxybutyric acid and its related compounds by gas chromatography-mass spectrometry in a patient with succinic semialdehyde dehydrogenase deficiency. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Aug 25;776(1):57-63. [PubMed:12127325 ]
  6. Minami T, Oomura Y, Nabekura J, Fukuda A: Direct effects of 3,4-dihydroxybutanoic acid gamma-lactone and 2,4,5-trihydroxypentanoic acid gamma-lactone on lateral and ventromedial hypothalamic neurons. Brain Res. 1988 Oct 18;462(2):258-64. [PubMed:3191387 ]
  7. Shimizu N, Oomura Y, Sakata T: Modulation of feeding by endogenous sugar acids acting as hunger or satiety factors. Am J Physiol. 1984 Apr;246(4 Pt 2):R542-50. doi: 10.1152/ajpregu.1984.246.4.R542. [PubMed:6720928 ]
  8. Mousavi M, Jonsson P, Antti H, Adolfsson R, Nordin A, Bergdahl J, Eriksson K, Moritz T, Nilsson LG, Nyberg L: Serum metabolomic biomarkers of dementia. Dement Geriatr Cogn Dis Extra. 2014 Jul 11;4(2):252-62. doi: 10.1159/000364816. eCollection 2014 May. [PubMed:25177334 ]
  9. Wang J, Shen X, Jain R, Wang J, Yuan Q, Yan Y: Establishing a novel biosynthetic pathway for the production of 3,4-dihydroxybutyric acid from xylose in Escherichia coli. Metab Eng. 2017 May;41:39-45. doi: 10.1016/j.ymben.2017.03.003. Epub 2017 Mar 23. [PubMed:28342964 ]