| Common Name |
2-Methyl-3-hydroxybutyric acid
| Description |
3-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 |
MOLSDF3D-SDFPDBSMILESInChI View 3D Structure
| Synonyms |
| Value |
Source |
2-Methyl-3-hydroxybutanoic acidChEBI
3-Hydroxy-2-methylbutyric acidChEBI
2-Methyl-3-hydroxybutanoateGenerator
2-Methyl-3-hydroxybutyrateGenerator
3-Hydroxy-2-methylbutyrateGenerator
3-Hydroxy-2-methyl-butyrateHMDB
3-Hydroxy-2-methyl-butyric acidHMDB
3-Hydroxy-2-methylbutanoateHMDB
3-Hydroxy-2-methylbutanoic acidHMDB
a-Methyl-b-hydroxybutyrateHMDB
a-Methyl-b-hydroxybutyric acidHMDB
alpha-Methyl-beta-hydroxybutyrateHMDB
alpha-Methyl-beta-hydroxybutyric acidHMDB
NilateHMDB
Nilic acidHMDB
2-Methyl-3-hydroxybutyric acid, (R-(r*,s*))-isomerMeSH
| Chemical Formlia |
C5H10O3
| Average Molecliar Weight |
118.1311
| Monoisotopic Molecliar Weight |
118.062994186
| IUPAC Name |
3-hydroxy-2-methylbutanoic acid
| Traditional Name |
nilic acid
| CAS Registry Number |
473-86-9
| 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 Key |
VEXDRERIMPLZLU-UHFFFAOYSA-N
| Chemical Taxonomy |
| Description |
This compound belongs to the class of chemical entities known as hydroxy fatty acids. These are fatty acids in which the chain bears a hydroxyl group.
| Kingdom |
Chemical entities
| Super Class |
Organic compounds
| Class |
Lipids and lipid-like moleclies
| Sub Class |
Fatty Acyls
| Direct Parent |
Hydroxy fatty acids
| Alternative Parents |
Short-chain hydroxy acids and derivatives
Methyl-branched fatty acids
Beta hydroxy acids and derivatives
Secondary alcohols
Monocarboxylic acids and derivatives
Carboxylic acids
Organic oxides
Hydrocarbon derivatives
Carbonyl compounds
| Substituents |
Beta-hydroxy acid
Branched fatty acid
Hydroxy fatty acid
Short-chain hydroxy acid
Methyl-branched fatty acid
Hydroxy acid
Secondary alcohol
Monocarboxylic acid or derivatives
Carboxylic acid
Carboxylic acid derivative
Organooxygen compound
Alcohol
Carbonyl group
Hydrocarbon derivative
Organic oxide
Organic oxygen compound
Aliphatic acyclic compound
| Molecliar Framework |
Aliphatic acyclic compounds
| External Descriptors |
3-hydroxy monocarboxylic acid (CHEBI:37051 )
Hydroxy fatty acids (LMFA01050391 )
| Ontology |
| Status |
Detected and Quantified
| Origin |
Endogenous
| Biofunction |
Not Available
| Application |
Not Available
| Cellliar locations |
Cytoplasm (predicted from logP)
| Physical Properties |
| State |
Solid
| Experimental Properties |
| Property |
Value |
Reference |
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
| Predicted Properties |
| Property |
Value |
Source |
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
Rlie of FiveYesChemAxon
Ghose FilterYesChemAxon
Vebers RlieYesChemAxon
MDDR-like RlieYesChemAxon
| Spectra |
| Spectra |
| Spectrum Type |
Description |
Splash Key |
|
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 10V, Positivesplash10-0uxr-3900000000-43c640882c221c34cff4View in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 20V, Positivesplash10-0zfr-9500000000-f67232e28fb95b61d1f2View in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 40V, Positivesplash10-0a4i-9000000000-da7c5873617fe20d6385View in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 10V, Negativesplash10-014i-5900000000-bab83930d1557839e6f7View in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 20V, Negativesplash10-05fr-9100000000-50701029b9e5b3af2fbfView in MoNA
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 40V, Negativesplash10-0a4i-9000000000-dd1dac699c164a7b71f5View in MoNA
| Biological Properties |
| Cellliar Locations |
Cytoplasm (predicted from logP)
| Biofluid Locations |
Urine
| Tissue Location |
Not Available
| Pathways |
Not Available
| Normal Concentrations |
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
UrineDetected but not Quantified Adlit (>18 years old)Both
Normal
24023812
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 creatinineAdlit (>18 years old)Both
Normal
24023812
details
UrineDetected and Quantified0.1 (0.1-4.1) umol/mmol creatinineNewborn (0-30 days old)BothNormal
8087979
details
UrineDetected and Quantified0.1 (0.1-5.9) umol/mmol creatinineInfant (0-1 year old)BothNormal
8087979
details
UrineDetected and Quantified1.5 (0.1-7.0) umol/mmol creatinineChildren (1-13 years old)Both
Normal
8087979
details
UrineDetected and Quantified1.7 (0.6-2.5) umol/mmol creatinineChildren (1-13 years old)Both
Normal
8087979
details
UrineDetected and Quantified1.2 (0.1-3.8) umol/mmol creatinineAdolescent (13-18 years old)Both
Normal
8087979
details
UrineDetected and Quantified4.0 (1.3-6.2) umol/mmol creatinineAdlit (>18 years old)Both
Normal
24023812
details
|
| Abnormal Concentrations |
|
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
UrineDetected and Quantified5.5 (0.0-11.0) umol/mmol creatinineAdlit (>18 years old)BothBeta-ketothiolase deficiency
MetaGene
details
UrineDetected and Quantified2300.0 (200.0-4400.0) umol/mmol creatinineChildren (1-13 years old)BothBeta-Ketothiolase deficiency
MetaGene
details
UrineDetected and Quantified105.00 (10.00-200.00) umol/mmol creatinineChildren (1-13 years old)BothKetosis
MetaGene
details
| Associated Disorders and Diseases |
| Disease References |
| Beta-ketothiolase deficiency |
- MetaGene [Link]
| Associated OMIM IDs |
203750 (Beta-ketothiolase deficiency)
| External Links |
| DrugBank ID |
Not Available
| DrugBank Metabolite ID |
Not Available
| Phenol Explorer Compound ID |
Not Available
| Phenol Explorer Metabolite ID |
Not Available
| FoodDB ID |
FDB021977
| KNApSAcK ID |
Not Available
| Chemspider ID |
141015
| KEGG Compound ID |
Not Available
| BioCyc ID |
Not Available
| BiGG ID |
Not Available
| Wikipedia Link |
Not Available
| NuGOwiki Link |
HMDB00354
| Metagene Link |
HMDB00354
| METLIN ID |
3786
| PubChem Compound |
160471
| PDB ID |
Not Available
| ChEBI ID |
37051
Product: Puerarin
References |
| Synthesis Reference |
Inoue, 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 |
- 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 ]
- 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. [PubMed:126826 ]
- 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. [PubMed:6467607 ]
- 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. [PubMed:10755375 ]
- Sutton VR, OBrien WE, Clark GD, Kim J, Wanders RJ: 3-Hydroxy-2-methylbutyryl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 2003;26(1):69-71. [PubMed:12872843 ]
|
PMID: 9262334