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Details

Stereochemistry RACEMIC
Molecular Formula C5H10O3
Molecular Weight 118.1311
Optical Activity ( + / - )
Defined Stereocenters 0 / 1
E/Z Centers 0
Charge 0

SHOW SMILES / InChI
Structure of 2-HYDROXYISOVALERIC ACID

SMILES

CC(C)C(O)C(O)=O

InChI

InChIKey=NGEWQZIDQIYUNV-UHFFFAOYSA-N
InChI=1S/C5H10O3/c1-3(2)4(6)5(7)8/h3-4,6H,1-2H3,(H,7,8)

HIDE SMILES / InChI
Molecular Formula C5H10O3
Molecular Weight 118.1311
Charge 0
Count
Stereochemistry RACEMIC
Additional Stereochemistry No
Defined Stereocenters 0 / 1
E/Z Centers 0
Optical Activity ( + / - )

Description

2-Hydroxyisovaleric acid (also known as 2-Hydroxy-3-methylbutyric acid) is a metabolite found in the urine patients with acidosis and diabetic ketoacidosis. The concentration of this metabolite was also abnormal high in the urine of severely asphyxiated group. In case of maple syrup urine disease, when also the concentration of the 2-hydroxyisovaleric acid was increased in the urine, was made a suggestion, that the early diagnosis and proper treatment can prevent brain damage and improve prognosis.

Approval Year

Unknown
149124
Conditions

Conditions

ConditionModalityTargetsHighest PhaseProduct
Diagnostic
Natural Metabolite
Unknown

Approved Use

Unknown
Diagnostic
Natural Metabolite
Unknown

Approved Use

Unknown
Diagnostic
Natural Metabolite
Unknown

Approved Use

Unknown
Diagnostic
Natural Metabolite
Unknown

Approved Use

Unknown
PubMed

PubMed

TitleDatePubMed
NMR-based metabonomic analysis of the hepatotoxicity induced by combined exposure to PCBs and TCDD in rats.
2010-11-01
Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain.
2010-04-09
Biosynthesis of the cyclooligomer depsipeptide bassianolide, an insecticidal virulence factor of Beauveria bassiana.
2009-05
Biosynthesis of the cyclooligomer depsipeptide beauvericin, a virulence factor of the entomopathogenic fungus Beauveria bassiana.
2008-09-22
Metabolic viability assessment of cystic echinococcosis using high-field 1H MRS of cyst contents.
2008-08
Proton HR-MAS NMR spectroscopic characterization of metabolites in various human organ tissues: pancreas, brain and liver from trauma cases.
2008
NMR-spectroscopy-based metabonomic approach to the analysis of Bay41-4109, a novel anti-HBV compound, induced hepatotoxicity in rats.
2007-09-28
Cytotoxic and Antihaptotactic beauvericin analogues from precursor-directed biosynthesis with the insect pathogen Beauveria bassiana ATCC 7159.
2007-09
Two step derivatization for the analyses of organic, amino acids and glycines on filter paper plasma by GC-MS/SIM.
2007-03
Pleofungins, novel inositol phosphorylceramide synthase inhibitors, from Phoma sp. SANK 13899. II. Structural elucidation.
2007-02
Absolute configuration of the creatonotines and callimorphines, two classes of arctiid-specific pyrrolizidine alkaloids.
2007-01
Multidimensional chromatographic approach applied to the identification of novel aroma compounds in wine. Identification of ethyl cyclohexanoate, ethyl 2-hydroxy-3-methylbutyrate and ethyl 2-hydroxy-4-methylpentanoate.
2006-12-29
Low-abundance kutznerides from Kutzneria sp. 744.
2006-12
Deciphering the biosynthetic codes for the potent anti-SARS-CoV cyclodepsipeptide valinomycin in Streptomyces tsusimaensis ATCC 15141.
2006-03
Intrahippocampal administration of the branched-chain alpha-hydroxy acids accumulating in maple syrup urine disease compromises rat performance in aversive and non-aversive behavioral tasks.
2005-05-15
Exceptional chiral recognition of racemic carboxylic acids by calix[4]arenes bearing optically pure alpha,beta-amino alcohol groups.
2004-04-15
Disturbances of valine metabolism in patients with peroxisomal biogenesis disorders.
2003
Enniatin synthetase is a monomer with extended structure: evidence for an intramolecular reaction mechanism.
2002-10
Sebastianines A and B, novel biologically active pyridoacridine alkaloids from the Brazilian ascidian Cystodytes dellechiajei.
2002-07-26
Directed biosynthesis of new enniatins.
2001-10
Enantiomeric purity determination of malic acid in apple juices by multi-beam circular dichroism detection.
2001-09-14
Gas chromatography-mass spectrometry with tert.-butyldimethylsilyl derivation: use of the simplified sample preparations and the automated data system to screen for organic acidemias.
2000-09-01
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.
1994-08-01
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.
1981-04
The occurrence of 2-hydroxyisovaleric acid in patients with lactic acidosis and ketoacidosis.
1977-08-01
Patents

Patents

08466133
2
08673908
6
08741877
2

Sample Use Guides

In Vivo Use Guide
Unknown
Route of Administration: Unknown
In Vitro Use Guide
Curator's Comment: Urinary 2-hydroxy isovaleric acid, 3-hydroxybutyric acid, 2-keto isovaleric acid, and 2-keto acid also increased. Both the blood amino acid and urine organic acid profiles led to the diagnosis of maple syrup urine disease.
Unknown
Substance Class Chemical
Created
by admin
on Mon Mar 31 21:57:02 GMT 2025
Edited
by admin
on Mon Mar 31 21:57:02 GMT 2025
Record UNII
02X1W97FWN
Record Status Validated (UNII)
Record Version
  • Download
Name Type Language
2-HYDROXYISOVALERIC ACID
Systematic Name English
NSC-227884
Preferred Name English
(±)-.ALPHA.-HYDROXYISOVALERIC ACID
Systematic Name English
2-HYDROXY-3-METHYLBUTANOIC ACID
Systematic Name English
3-METHYL-2-HYDROXYBUTYRIC ACID
Systematic Name English
DL-2-HYDROXY-3-METHYLBUTANOIC ACID
Systematic Name English
DL-2-HYDROXYISOVALERIC ACID
Systematic Name English
BUTANOIC ACID, 2-HYDROXY-3-METHYL-
Systematic Name English
(±)-2-HYDROXYISOPENTANOIC ACID
Systematic Name English
(±)-2-HYDROXY-3-METHYLBUTYRIC ACID
Systematic Name English
DL-.ALPHA.-HYDROXYISOVALERIC ACID
Systematic Name English
2-OXYISOVALERIC ACID
Systematic Name English
.ALPHA.-HYDROXYISOVALERIC ACID
Systematic Name English
2-HYDROXY-3-METHYLBUTYRIC ACID
Systematic Name English
BUTYRIC ACID, 2-HYDROXY-3-METHYL-
Systematic Name English
(±)-2-HYDROXYISOVALERIC ACID
Systematic Name English
Code System Code Type Description
PUBCHEM
99823
Created by admin on Mon Mar 31 21:57:02 GMT 2025 , Edited by admin on Mon Mar 31 21:57:02 GMT 2025
PRIMARY
FDA UNII
02X1W97FWN
Created by admin on Mon Mar 31 21:57:02 GMT 2025 , Edited by admin on Mon Mar 31 21:57:02 GMT 2025
PRIMARY
NSC
227884
Created by admin on Mon Mar 31 21:57:02 GMT 2025 , Edited by admin on Mon Mar 31 21:57:02 GMT 2025
PRIMARY
CHEBI
60645
Created by admin on Mon Mar 31 21:57:02 GMT 2025 , Edited by admin on Mon Mar 31 21:57:02 GMT 2025
PRIMARY
CAS
4026-18-0
Created by admin on Mon Mar 31 21:57:02 GMT 2025 , Edited by admin on Mon Mar 31 21:57:02 GMT 2025
PRIMARY
EPA CompTox
DTXSID10863305
Created by admin on Mon Mar 31 21:57:02 GMT 2025 , Edited by admin on Mon Mar 31 21:57:02 GMT 2025
PRIMARY
ECHA (EC/EINECS)
223-697-5
Created by admin on Mon Mar 31 21:57:02 GMT 2025 , Edited by admin on Mon Mar 31 21:57:02 GMT 2025
PRIMARY
Related Record Type Details
Structure of 2-HYDROXYISOVALERIC ACID, (S)-
ENANTIOMER -> RACEMATE
Structure of 2-HYDROXYISOVALERIC ACID, (R)-
ENANTIOMER -> RACEMATE
NIH
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