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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-06-10 21:21:55 UTC
HMDB IDHMDB00407
Secondary Accession NumbersNone
Metabolite Identification
Common Name2-Hydroxy-3-methylbutyric acid
Description2-Hydroxy-3-methylbutyric acid (also known as 2-Hydroxyisovaleric acid) is a metabolite found in the urine of patients with Phenylketonuria (PMID 7978272 ), Methylmalonic acidemia, Propionic acidemia, 3-Ketothiolase deficiency, Isovaleric acidemia, 3-Methylcrotonylglycemia, 3-Hydroxy-3-methylglutaric acidemia, Multiple carboxylase deficiency, Glutaric aciduria, Ornithine transcarbamylase deficiency, glyceroluria, Tyrosinemia type 1, Galactosemia, and Maple syrup urine disease (PMID 11048741 ). 2-hydroxyisovaleric acid has been identified in the urine of patients with lactic and ketoacidosis (PMID: 884872 ). It has also been identified in the urine of severely asphyxiated babies (PMID: 1610944 ). 2-hydroxyisovaleric acid originates mainly from ketogenesis and from the metabolism of valine, leucine and isoleucine (PMID: 6434570 ).
Structure
Thumb
Synonyms
ValueSource
2-Hydroxyisopentanoic acidChEBI
2-Hydroxyisovaleric acidChEBI
3-Methyl-2-hydroxybutyric acidChEBI
alpha-Hydroxyisovaleric acidChEBI
2-HydroxyisopentanoateGenerator
2-Hydroxy-3-methylbutyrateGenerator
2-HydroxyisovalerateGenerator
3-Methyl-2-hydroxybutyrateGenerator
a-HydroxyisovalerateGenerator
a-Hydroxyisovaleric acidGenerator
alpha-HydroxyisovalerateGenerator
α-hydroxyisovalerateGenerator
α-hydroxyisovaleric acidGenerator
2-Hydroxy-3-methyl-butyric acidHMDB
2-Hydroxy-3-methylbutanoateHMDB
2-Hydroxy-3-methylbutanoic acidHMDB
2-OxyisovalerateHMDB
2-Oxyisovaleric acidHMDB
DL-2-Hydroxy-3-methylbutanoateHMDB
DL-2-Hydroxy-3-methylbutanoic acidHMDB
DL-2-HydroxyisovalerateHMDB
DL-2-Hydroxyisovaleric acidHMDB
DL-a-HydroxyisovalerateHMDB
DL-a-Hydroxyisovaleric acidHMDB
DL-alpha-HydroxyisovalerateHMDB
DL-alpha-Hydroxyisovaleric acidHMDB
Chemical FormulaC5H10O3
Average Molecular Weight118.1311
Monoisotopic Molecular Weight118.062994186
IUPAC Name2-hydroxy-3-methylbutanoic acid
Traditional Name2-hydroxyisovaleric acid
CAS Registry Number4026-18-0
SMILES
CC(C)C(O)C(O)=O
InChI Identifier
InChI=1S/C5H10O3/c1-3(2)4(6)5(7)8/h3-4,6H,1-2H3,(H,7,8)
InChI KeyInChIKey=NGEWQZIDQIYUNV-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
  • Branched fatty acid
  • Hydroxy acid
  • Alpha-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
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Cytoplasm
  • Extracellular
  • Membrane
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility350 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility215.0 mg/mLALOGPS
logP0.1ALOGPS
logP0.42ChemAxon
logS0.26ALOGPS
pKa (Strongest Acidic)4.14ChemAxon
pKa (Strongest Basic)-3.9ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity27.84 m3·mol-1ChemAxon
Polarizability11.77 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MSNot Available
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Negative (Annotated)splash10-01b9-9800000000-34c80f5b3cfe512db815View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Negative (Annotated)splash10-006t-9100000000-62b5dd0328186489f167View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Negative (Annotated)splash10-00kb-9400000000-4b607be60b20b11a9328View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, NegativeNot Available
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified4.5 (2.9-6.2) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified7.7 (0.0-19.0) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified8 +/- 6 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified6.8 +/- 6.8 uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified3.13 +/- 0.24 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
UrineDetected and Quantified0.21-0.48 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified0.0 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified2.0 (0.0-4.5) umol/mmol creatinineInfant (0-1 year old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified0.23-0.42 umol/mmol creatinineAdult (>18 years old)MaleNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified2.375 +/- 2.017 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
UrineDetected and Quantified2225.00 (850.00-3600.00) umol/mmol creatinineChildren (1-13 years old)BothMaple syrup urine disease (MSUD) details
Associated Disorders and Diseases
Disease References
Maple syrup urine disease
  1. MetaGene [Link]
Associated OMIM IDs
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022023
KNApSAcK IDNot Available
Chemspider ID90190
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00407
Metagene LinkHMDB00407
METLIN ID5396
PubChem Compound99823
PDB IDNot Available
ChEBI ID60645
References
Synthesis ReferencePerekalin, V. V.; Petryaeva, A. K.; Zobacheva, M. M.; Metelkina, E. L. A new method of synthesis of a-hydroxy and a-oxo acids. Doklady Akademii Nauk SSSR (1966), 166(5), 1129-31.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Bergstrom T, Greter J, Levin AH, Steen G, Tryding N, Wass U: Propionyl-CoA carboxylase deficiency: case report, effect of low-protein diet and identification of 3-oxo-2-methylvaleric acid 3-hydroxy-2-methylvaleric acid, and maleic acid in urine. Scand J Clin Lab Invest. 1981 Apr;41(2):117-26. [7313494 ]
  2. Landaas S, Jakobs C: The occurrence of 2-hydroxyisovaleric acid in patients with lactic acidosis and ketoacidosis. Clin Chim Acta. 1977 Aug 1;78(3):489-93. [884872 ]
  3. Magni F, Arnoldi L, Galati G, Galli Kienle M: Simultaneous determination of plasma levels of alpha-ketoisocaproic acid and leucine and evaluation of alpha-[1-13C]ketoisocaproic acid and [1-13C]leucine enrichment by gas chromatography-mass spectrometry. Anal Biochem. 1994 Aug 1;220(2):308-14. [7978272 ]
  4. Ohie T, Fu X, Iga M, Kimura M, Yamaguchi S: Gas chromatography-mass spectrometry with tert.-butyldimethylsilyl derivation: use of the simplified sample preparations and the automated data system to screen for organic acidemias. J Chromatogr B Biomed Sci Appl. 2000 Sep 1;746(1):63-73. [11048741 ]
  5. Liebich HM, Forst C: Hydroxycarboxylic and oxocarboxylic acids in urine: products from branched-chain amino acid degradation and from ketogenesis. J Chromatogr. 1984 Aug 10;309(2):225-42. [6434570 ]
  6. Walker V, Mills GA: Effects of birth asphyxia on urinary organic acid excretion. Biol Neonate. 1992;61(3):162-72. [1610944 ]