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
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:02:34 UTC
HMDB IDHMDB00337
Secondary Accession Numbers
  • HMDB06118
Metabolite Identification
Common Name(S)-3,4-Dihydroxybutyric acid
Description3,4-Dihydroxybutyric acid is a normal human urinary metabolite that is excreted in increased concentration in patients with succinic semialdehyde dehydrogenase (SSADH) deficiency. (PMID 12127325 ) Normal adults excrete 0.37 +/- 0.15 (SD) mmoles of 3,4-dihydroxybutyrate per 24 hr. The compound is also detectable in blood. The renal clearance of this compound is roughly equal that of creatinine.
Structure
Thumb
Synonyms
ValueSource
(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
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 KeyInChIKey=DZAIOXUZHHTJKN-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentHydroxy fatty acids
Alternative Parents
Substituents
  • Hydroxy fatty acid
  • Short-chain hydroxy acid
  • Beta-hydroxy acid
  • Hydroxy acid
  • Secondary alcohol
  • 1,2-diol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Primary alcohol
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locationsNot Available
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 Solubility832.0 mg/mLALOGPS
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 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity25.01 m3·mol-1ChemAxon
Polarizability10.91 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0udr-5900000000-87e855dfa4a5229ec106View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0gw0-9300000000-88020d54d9b37645b873View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4u-9000000000-d0c64e75e2e9de34f683View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-7900000000-6aec80cde1bf2e0bca19View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0pxr-9300000000-93d09801aa0c902a4e8fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9000000000-d765ea0631d195240881View in MoNA
Biological Properties
Cellular LocationsNot Available
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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 ApplicableNot SpecifiedNot Specified
Normal
details
FecesDetected but not QuantifiedNot ApplicableInfant (0-1 year old)Both
Normal
details
UrineDetected but not QuantifiedNot ApplicableAdult (>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 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
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified98.0 (30.0-166.0) umol/mmol creatinineAdult (>18 years old)BothSuccinic semialdehyde dehydrogenase deficiency details
UrineDetected and Quantified137.5 (50.0-225.0) umol/mmol creatinineChildren (1-13 years old)Both4-Hydroxybutyric Aciduria details
Associated Disorders and Diseases
Disease References
Succinic semialdehyde dehydrogenase deficiency
  1. MetaGene [Link]
  2. MetaGene [Link]
Associated OMIM IDs
  • 271980 (Succinic semialdehyde dehydrogenase deficiency)
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021961
KNApSAcK IDNot Available
Chemspider ID133026
KEGG Compound IDNot Available
BioCyc ID3-4DH3-4DHFLUORENE
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00337
Metagene LinkHMDB00337
METLIN IDNot Available
PubChem Compound150929
PDB IDNot Available
ChEBI 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. [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. [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. [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. [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. [12127325 ]