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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:02:35 UTC
HMDB IDHMDB00354
Secondary Accession Numbers
  • HMDB00420
  • HMDB00605
  • HMDB02187
Metabolite Identification
Common Name2-Methyl-3-hydroxybutyric acid
Description3-Hydroxy-2-methyl-butanoic acid (HMBA) is a normal urinary metabolite involved in the isoleucine catabolism, as well as presumably beta-oxidation of fatty acids and ketogenesis, excreted in abnormally high amounts in beta-ketothiolase deficiency. Differences in the enantiomeric ratio of HMBA may originate from the enantioselectivity of different enzyme systems (PMID 10755375 ).
Structure
Thumb
Synonyms
ValueSource
3-Hydroxy-2-methylbutyric acidChEBI
3-Hydroxy-2-methylbutyrateGenerator
3-Hydroxy-2-methylbutanoateGenerator
2-Methyl-3-hydroxybutanoateHMDB
2-Methyl-3-hydroxybutanoic acidHMDB
2-Methyl-3-hydroxybutyrateHMDB
3-Hydroxy-2-methyl-butyrateHMDB
3-Hydroxy-2-methyl-butyric acidHMDB
a-Methyl-b-hydroxybutyrateHMDB
a-Methyl-b-hydroxybutyric acidHMDB
alpha-Methyl-beta-hydroxybutyrateHMDB
alpha-Methyl-beta-hydroxybutyric acidHMDB
NilateHMDB
Nilic acidHMDB
Chemical FormulaC5H10O3
Average Molecular Weight118.1311
Monoisotopic Molecular Weight118.062994186
IUPAC Name3-hydroxy-2-methylbutanoic acid
Traditional Namenilic acid
CAS Registry Number473-86-9
SMILES
CC(O)C(C)C(O)=O
InChI Identifier
InChI=1S/C5H10O3/c1-3(4(2)6)5(7)8/h3-4,6H,1-2H3,(H,7,8)
InChI KeyInChIKey=VEXDRERIMPLZLU-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
  • Methyl-branched fatty acid
  • Short-chain hydroxy acid
  • Branched fatty acid
  • Beta-hydroxy acid
  • Hydroxy acid
  • Secondary alcohol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • 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 locations
  • Cytoplasm (predicted from logP)
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 Solubility336.0 mg/mLALOGPS
logP-0.13ALOGPS
logP0.16ChemAxon
logS0.45ALOGPS
pKa (Strongest Acidic)4.51ChemAxon
pKa (Strongest Basic)-2.8ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity28.04 m3·mol-1ChemAxon
Polarizability11.83 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, Positivesplash10-0uxr-3900000000-43c640882c221c34cff4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0zfr-9500000000-f67232e28fb95b61d1f2View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4i-9000000000-da7c5873617fe20d6385View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-014i-5900000000-bab83930d1557839e6f7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-05fr-9100000000-50701029b9e5b3af2fbfView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4i-9000000000-dd1dac699c164a7b71f5View in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified5.0 (1.0-9.0) umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified<30 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified4.2 (1.6-6.7) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.1 (0.1-4.1) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified0.1 (0.1-5.9) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified1.5 (0.1-7.0) umol/mmol creatinineChildren (1-13 years old)Both
Normal
details
UrineDetected and Quantified1.7 (0.6-2.5) umol/mmol creatinineChildren (1-13 years old)Both
Normal
details
UrineDetected and Quantified1.2 (0.1-3.8) umol/mmol creatinineAdolescent (13-18 years old)Both
Normal
details
UrineDetected and Quantified4.0 (1.3-6.2) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified5.5 (0.0-11.0) umol/mmol creatinineAdult (>18 years old)BothBeta-ketothiolase deficiency details
UrineDetected and Quantified2300.0 (200.0-4400.0) umol/mmol creatinineChildren (1-13 years old)BothBeta-Ketothiolase deficiency details
UrineDetected and Quantified105.00 (10.00-200.00) umol/mmol creatinineChildren (1-13 years old)BothKetosis details
Associated Disorders and Diseases
Disease References
Beta-ketothiolase deficiency
  1. MetaGene [Link]
Associated OMIM IDs
  • 203750 (Beta-ketothiolase deficiency)
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB021977
KNApSAcK IDNot Available
Chemspider ID141015
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00354
Metagene LinkHMDB00354
METLIN ID3786
PubChem Compound160471
PDB IDNot Available
ChEBI ID37051
References
Synthesis ReferenceInoue, Yoshio; Sano, Fumitaka; Nakamura, Kazuhiro; Yoshie, Naoko; Saito, Yuji; Satoh, Hiroyasu; Mino, Takashi; Matsuo, Tomonori; Doi, Yoshiharu. Microstructure of copoly(3-hydroxyalkanoates) produced in the anaerobic-aerobic activated sludge process. Polymer International (1996), 39(3), 183-9.
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. Landaas S: Accumulation of 3-hydroxyisobutyric acid, 2-methyl-3-hydroxybutyric acid and 3-hydroxyisovaleric acid in ketoacidosis. Clin Chim Acta. 1975 Oct 15;64(2):143-54. [126826 ]
  3. Jakobs C, Sweetman L, Nyhan WL: Hydroxy acid metabolites of branched-chain amino acids in amniotic fluid. Clin Chim Acta. 1984 Jul 16;140(2):157-66. [6467607 ]
  4. Heil M, Podebrad F, Prado E, Beck T, Mosand A, Sewell AC, Bohles H, Lehnert W: Enantioselective analysis of ketone bodies in patients with beta-ketothiolase deficiency, medium-chain acyl coenzyme A dehydrogenase deficiency and ketonemic vomiting. J Chromatogr B Biomed Sci Appl. 2000 Mar 10;739(2):313-24. [10755375 ]
  5. Sutton VR, O'Brien WE, Clark GD, Kim J, Wanders RJ: 3-Hydroxy-2-methylbutyryl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 2003;26(1):69-71. [12872843 ]