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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:14:48 UTC
HMDB IDHMDB0000407
Secondary Accession Numbers
  • HMDB00407
  • HMDB0062584
  • HMDB62584
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 I, galactosemia, and maple syrup urine disease (PMID: 11048741 ). 2-Hydroxyisovaleric acid has also been identified in the urine of patients with lactic acidosis and ketoacidosis (PMID: 884872 ), and 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 ). 2-Hydroxy-3-methylbutyric acid has been identified in the human placenta (PMID: 32033212 ).
Structure
Data?1676999688
Synonyms
ValueSource
2-Hydroxyisopentanoic acidChEBI
2-Hydroxyisovaleric acidChEBI
3-Methyl-2-hydroxybutyric acidChEBI
alpha-Hydroxyisovaleric acidChEBI
2-HydroxyisopentanoateGenerator
2-HydroxyisovalerateGenerator
3-Methyl-2-hydroxybutyrateGenerator
a-HydroxyisovalerateGenerator
a-Hydroxyisovaleric acidGenerator
alpha-HydroxyisovalerateGenerator
Α-hydroxyisovalerateGenerator
Α-hydroxyisovaleric acidGenerator
2-Hydroxy-3-methylbutyrateGenerator
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
2-Hydroxyisovaleric acid, monosodium salt, (S)-isomerHMDB
2-Hydroxyisovaleric acid, (S)-isomerHMDB
2-Hydroxyisovaleric acid, calcium (2:1) saltHMDB
2-Hydroxyisovaleric acid, (R)-isomerHMDB
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 KeyNGEWQZIDQIYUNV-UHFFFAOYSA-N
Chemical Taxonomy
Description 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
  • Branched fatty acid
  • Hydroxy fatty acid
  • Methyl-branched fatty acid
  • Alpha-hydroxy acid
  • Hydroxy acid
  • Secondary alcohol
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Hydrocarbon derivative
  • Organic oxide
  • Organic oxygen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Biological locationRoute of exposureSource
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility350 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-MetCCS_train_neg118.64930932474
[M-H]-Not Available118.649http://allccs.zhulab.cn/database/detail?ID=AllCCS00000192
Predicted Molecular Properties
PropertyValueSource
Water Solubility215 g/LALOGPS
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 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity27.84 m³·mol⁻¹ChemAxon
Polarizability11.77 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+125.0231661259
DarkChem[M-H]-119.05831661259
AllCCS[M+H]+129.52532859911
AllCCS[M-H]-125.91232859911
DeepCCS[M+H]+127.76930932474
DeepCCS[M-H]-124.9730932474
DeepCCS[M-2H]-161.39930932474
DeepCCS[M+Na]+136.10330932474
AllCCS[M+H]+129.532859911
AllCCS[M+H-H2O]+125.432859911
AllCCS[M+NH4]+133.432859911
AllCCS[M+Na]+134.532859911
AllCCS[M-H]-125.932859911
AllCCS[M+Na-2H]-129.232859911
AllCCS[M+HCOO]-132.932859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
2-Hydroxy-3-methylbutyric acidCC(C)C(O)C(O)=O2002.9Standard polar33892256
2-Hydroxy-3-methylbutyric acidCC(C)C(O)C(O)=O986.2Standard non polar33892256
2-Hydroxy-3-methylbutyric acidCC(C)C(O)C(O)=O1046.1Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
2-Hydroxy-3-methylbutyric acid,1TMS,isomer #1CC(C)C(O[Si](C)(C)C)C(=O)O1146.4Semi standard non polar33892256
2-Hydroxy-3-methylbutyric acid,1TMS,isomer #2CC(C)C(O)C(=O)O[Si](C)(C)C1039.3Semi standard non polar33892256
2-Hydroxy-3-methylbutyric acid,2TMS,isomer #1CC(C)C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1190.6Semi standard non polar33892256
2-Hydroxy-3-methylbutyric acid,1TBDMS,isomer #1CC(C)C(O[Si](C)(C)C(C)(C)C)C(=O)O1376.7Semi standard non polar33892256
2-Hydroxy-3-methylbutyric acid,1TBDMS,isomer #2CC(C)C(O)C(=O)O[Si](C)(C)C(C)(C)C1262.8Semi standard non polar33892256
2-Hydroxy-3-methylbutyric acid,2TBDMS,isomer #1CC(C)C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1608.5Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxy-3-methylbutyric acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-006x-9100000000-3ceb85540106a01209a92016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxy-3-methylbutyric acid GC-MS (2 TMS) - 70eV, Positivesplash10-00g1-9520000000-27e47b254facef447ecf2017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxy-3-methylbutyric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxy-3-methylbutyric acid GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxy-3-methylbutyric acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxy-3-methylbutyric acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxy-3-methylbutyric acid GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxy-3-methylbutyric acid GC-MS (TBDMS_2_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid Quattro_QQQ 10V, Negative-QTOF (Annotated)splash10-01b9-9800000000-34c80f5b3cfe512db8152012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid Quattro_QQQ 25V, Negative-QTOF (Annotated)splash10-006t-9100000000-62b5dd0328186489f1672012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid Quattro_QQQ 40V, Negative-QTOF (Annotated)splash10-00kb-9400000000-4b607be60b20b11a93282012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid LC-ESI-IT , negative-QTOFsplash10-00di-9000000000-053084615922d2811fc02017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid Linear Ion Trap , negative-QTOFsplash10-00di-9000000000-c74232c34d7c991de0c52017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 35V, Negative-QTOFsplash10-052f-9000000000-1837dbe9ec7e7648abac2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 35V, Positive-QTOFsplash10-0gbc-9400000000-3dae3f739ba9de64475a2021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 10V, Positive-QTOFsplash10-0gi0-6900000000-9c5d211acf2118d6a1362016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 20V, Positive-QTOFsplash10-00dl-9100000000-fd55205401dd434cfdcc2016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 40V, Positive-QTOFsplash10-05fu-9000000000-44a71cd407c65a176f7d2016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 10V, Negative-QTOFsplash10-01b9-6900000000-977b23edb76b18575dc22016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 20V, Negative-QTOFsplash10-00xr-9400000000-a265e0ef4d3a3366c0be2016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 40V, Negative-QTOFsplash10-05fr-9000000000-a258a01f539f2e2cd1e72016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 10V, Positive-QTOFsplash10-0ab9-9100000000-4440e0dd7c3edf6166ae2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 20V, Positive-QTOFsplash10-0a4i-9000000000-848921fdd36001ccc2ad2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 40V, Positive-QTOFsplash10-0006-9000000000-ec34045be03977dd8e792021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 10V, Negative-QTOFsplash10-014i-2900000000-d5f27e5fc2f84d237f6a2021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 20V, Negative-QTOFsplash10-014j-7900000000-d8e0dd0fbaecb6b1909e2021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Hydroxy-3-methylbutyric acid 40V, Negative-QTOFsplash10-0a4i-9000000000-a096d70c21dd426764cb2021-09-23Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)2023-02-03FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified13.7 +/- 6.3 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Female
Normal
details
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 Quantified6.6 +/- 2.3 uMNewborn (0-30 days old)Not SpecifiedNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified6.8 +/- 6.8 uMAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>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 QuantifiedAdult (>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
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified6.9 (4.0) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
BloodDetected and Quantified4.1 (2.8) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified1237 uMInfant (0-1 year old)FemaleMaple syrup urine disease details
Cerebrospinal Fluid (CSF)Detected and Quantified809 uMNewborn (0-30 days old)FemaleMaple syrup urine disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified2.375 +/- 2.017 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
UrineDetected and Quantified407.016 umol/mmol creatinineInfant (0-1 year old)MaleDihydrolipoamide Dehydrogenase Deficiency details
Associated Disorders and Diseases
Disease References
Pregnancy
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ]
Maple syrup urine disease
  1. Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9. [PubMed:6422161 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Dihydrolipoamide Dehydrogenase Deficiency
  1. Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]
Associated OMIM IDs
  • 248600 (Maple syrup urine disease)
  • 114500 (Colorectal cancer)
  • 610247 (Eosinophilic esophagitis)
  • 246900 (Dihydrolipoamide Dehydrogenase Deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022023
KNApSAcK IDNot Available
Chemspider ID90190
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID5396
PubChem Compound99823
PDB IDNot Available
ChEBI ID60645
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000158
Good Scents IDNot Available
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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed:6434570 ]
  6. Walker V, Mills GA: Effects of birth asphyxia on urinary organic acid excretion. Biol Neonate. 1992;61(3):162-72. [PubMed:1610944 ]
  7. Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
  8. Watson AD: Thematic review series: systems biology approaches to metabolic and cardiovascular disorders. Lipidomics: a global approach to lipid analysis in biological systems. J Lipid Res. 2006 Oct;47(10):2101-11. Epub 2006 Aug 10. [PubMed:16902246 ]
  9. Sethi JK, Vidal-Puig AJ: Thematic review series: adipocyte biology. Adipose tissue function and plasticity orchestrate nutritional adaptation. J Lipid Res. 2007 Jun;48(6):1253-62. Epub 2007 Mar 20. [PubMed:17374880 ]
  10. Lingwood D, Simons K: Lipid rafts as a membrane-organizing principle. Science. 2010 Jan 1;327(5961):46-50. doi: 10.1126/science.1174621. [PubMed:20044567 ]
  11. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]
  12. Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.

Enzymes

General function:
Involved in sulfotransferase activity
Specific function:
Sulfotransferase that utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) as sulfonate donor to catalyze the sulfate conjugation of many hormones, neurotransmitters, drugs and xenobiotic compounds. Sulfonation increases the water solubility of most compounds, and therefore their renal excretion, but it can also result in bioactivation to form active metabolites. Sulfates hydroxysteroids like DHEA. Isoform 1 preferentially sulfonates cholesterol, and isoform 2 avidly sulfonates pregnenolone but not cholesterol.
Gene Name:
SULT2B1
Uniprot ID:
O00204
Molecular weight:
39598.595
Reactions
2-Hydroxy-3-methylbutyric acid → 3-methyl-2-(sulfooxy)butanoic aciddetails
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate. Is also able to catalyze the glucuronidation of 17beta-estradiol, 17alpha-ethinylestradiol, 1-hydroxypyrene, 4-methylumbelliferone, 1-naphthol, paranitrophenol, scopoletin, and umbelliferone.
Gene Name:
UGT1A1
Uniprot ID:
P22309
Molecular weight:
59590.91
Reactions
2-Hydroxy-3-methylbutyric acid → 6-(1-carboxy-2-methylpropoxy)-3,4,5-trihydroxyoxane-2-carboxylic aciddetails
2-Hydroxy-3-methylbutyric acid → 3,4,5-trihydroxy-6-[(2-hydroxy-3-methylbutanoyl)oxy]oxane-2-carboxylic aciddetails