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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:14:27 UTC
HMDB IDHMDB0000023
Secondary Accession Numbers
  • HMDB0000435
  • HMDB00023
  • HMDB00435
  • HMDB0062640
  • HMDB0126088
  • HMDB62640
Metabolite Identification
Common Name(S)-3-Hydroxyisobutyric acid
Description(S)-3-Hydroxyisobutyric acid (3-HIBA) (CAS: 2068-83-9) is an organic acid. 3-HIBA is an intermediate in L-valine metabolism. 3-HIBA plays an important role in the diagnosis of the very rare inherited metabolic diseases 3-hydroxyisobutyric aciduria (OMIM: 236795 ) and methylmalonic semialdehyde dehydrogenase deficiency (OMIM: 603178 ). Patients with 3-hydroxyisobutyric aciduria excrete a significant amount of 3-HIBA not only during the acute stage but also when stable. 3-Hydroxyisobutyric aciduria is caused by a 3-hydroxyisobutyryl-CoA dehydrogenase deficiency (PMID: 18329219 ). The severity of this disease varies from case to case. Most patients exhibit dysmorphic features, such as a small triangular face, a long philtrum, low set ears, and micrognathia (PMID: 10686279 ). Lactic acidemia is also found in the affected patients, indicating that mitochondrial dysfunction is involved. 3-HIBA appears to specifically inhibit the function of the respiratory chain complex I-III and mitochondrial creatine kinase (PMID: 18329219 ). BioTransformer predicts that 3-HIBA is a product of 2-methylpropanoic acid metabolism via a hydroxylation-of-terminal-methyl reaction catalyzed by CYP2B6 and CYP2E1 enzymes (PMID: 30612223 ).
Structure
Data?1676999667
Synonyms
ValueSource
(S)-3-Hydroxy-2-methylpropanoic acidChEBI
(S)-3-Hydroxy-2-methylpropionic acidChEBI
(S)-3-Hydroxy-2-methylpropanoateGenerator
(S)-3-Hydroxy-2-methylpropionateGenerator
(S)-3-HydroxyisobutyrateGenerator
2-Methyl-L-(+)-hydracrylateHMDB
2-Methyl-L-(+)-hydracrylic acidHMDB
3-Hydroxy(iso)butyric acidHMDB
3-Hydroxy-2-methyl-(S)-propanoateHMDB
3-Hydroxy-2-methyl-(S)-propanoic acidHMDB
3-Hydroxy-2-methylpropanoateHMDB
3-Hydroxy-2-methylpropanoic acidHMDB
3-Hydroxy-isobutyrateHMDB
3-HydroxyisobutyrateHMDB
3-Hydroxyisobutyric acidHMDB
(2S)-3-Hydroxy-2-methylpropanoic acidHMDB
(2S)-3-Hydroxy-2-methylpropionic acidHMDB
(S)-beta-Hydroxyisobutyric acidHMDB
(S)-Β-hydroxyisobutyric acidHMDB
(±)-3-hydroxy-2-methylpropanoic acidHMDB
(±)-3-hydroxy-2-methylpropionic acidHMDB
2-(Hydroxymethyl)propanoic acidHMDB
2-(Hydroxymethyl)propionic acidHMDB
2-Methyl-3-hydroxypropanoic acidHMDB
2-Methyl-3-hydroxypropionic acidHMDB
3-HIBAHMDB
3-Hydroxy-2-methylpropionic acidHMDB
DL-3-Hydroxyisobutyric acidHMDB
L-(+)-beta-Hydroxyisobutyric acidHMDB
L-(+)-Β-hydroxyisobutyric acidHMDB
beta-Hydroxyisobutyric acidHMDB
Β-hydroxyisobutyric acidHMDB
3-Hydroxy-2-isobutyrateHMDB
3-Hydroxy-2-isobutyric acidHMDB
(S)-3-Hydroxyisobutyric acidHMDB
Chemical FormulaC4H8O3
Average Molecular Weight104.1045
Monoisotopic Molecular Weight104.047344122
IUPAC Name(2S)-3-hydroxy-2-methylpropanoic acid
Traditional Name(S)-3-hydroxyisobutyric acid
CAS Registry Number26543-05-5
SMILES
C[C@@H](CO)C(O)=O
InChI Identifier
InChI=1S/C4H8O3/c1-3(2-5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1
InChI KeyDBXBTMSZEOQQDU-VKHMYHEASA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as beta hydroxy acids and derivatives. Beta hydroxy acids and derivatives are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassBeta hydroxy acids and derivatives
Direct ParentBeta hydroxy acids and derivatives
Alternative Parents
Substituents
  • Beta-hydroxy acid
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Primary alcohol
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Disposition
Biological locationSource
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility571 g/LALOGPS
logP-0.47ALOGPS
logP-0.26ChemAxon
logS0.74ALOGPS
pKa (Strongest Acidic)4.37ChemAxon
pKa (Strongest Basic)-2.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area57.53 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity23.62 m³·mol⁻¹ChemAxon
Polarizability9.98 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]+121.12431661259
DarkChem[M-H]-115.65331661259
AllCCS[M+H]+126.80532859911
AllCCS[M-H]-124.49432859911
DeepCCS[M+H]+118.73230932474
DeepCCS[M-H]-115.92230932474
DeepCCS[M-2H]-152.40230932474
DeepCCS[M+Na]+127.1730932474
AllCCS[M+H]+126.832859911
AllCCS[M+H-H2O]+122.532859911
AllCCS[M+NH4]+130.832859911
AllCCS[M+Na]+132.032859911
AllCCS[M-H]-124.532859911
AllCCS[M+Na-2H]-128.432859911
AllCCS[M+HCOO]-132.832859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
(S)-3-Hydroxyisobutyric acidC[C@@H](CO)C(O)=O2050.5Standard polar33892256
(S)-3-Hydroxyisobutyric acidC[C@@H](CO)C(O)=O932.7Standard non polar33892256
(S)-3-Hydroxyisobutyric acidC[C@@H](CO)C(O)=O1017.4Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
(S)-3-Hydroxyisobutyric acid,1TMS,isomer #1C[C@@H](CO[Si](C)(C)C)C(=O)O1085.9Semi standard non polar33892256
(S)-3-Hydroxyisobutyric acid,1TMS,isomer #2C[C@@H](CO)C(=O)O[Si](C)(C)C1020.5Semi standard non polar33892256
(S)-3-Hydroxyisobutyric acid,2TMS,isomer #1C[C@@H](CO[Si](C)(C)C)C(=O)O[Si](C)(C)C1170.4Semi standard non polar33892256
(S)-3-Hydroxyisobutyric acid,1TBDMS,isomer #1C[C@@H](CO[Si](C)(C)C(C)(C)C)C(=O)O1328.6Semi standard non polar33892256
(S)-3-Hydroxyisobutyric acid,1TBDMS,isomer #2C[C@@H](CO)C(=O)O[Si](C)(C)C(C)(C)C1255.6Semi standard non polar33892256
(S)-3-Hydroxyisobutyric acid,2TBDMS,isomer #1C[C@@H](CO[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1592.3Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - (S)-3-Hydroxyisobutyric acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-0ab9-9000000000-dd1c22f7a337d32894972016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - (S)-3-Hydroxyisobutyric acid GC-MS (2 TMS) - 70eV, Positivesplash10-00gr-9720000000-2a61310303ff8f2a7b422017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - (S)-3-Hydroxyisobutyric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - (S)-3-Hydroxyisobutyric acid GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - (S)-3-Hydroxyisobutyric acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - (S)-3-Hydroxyisobutyric acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - (S)-3-Hydroxyisobutyric acid GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - (S)-3-Hydroxyisobutyric 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 - (S)-3-Hydroxyisobutyric acid Quattro_QQQ 10V, Negative-QTOF (Annotated)splash10-0zfr-8900000000-b51c12e744025b833f312012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid Quattro_QQQ 25V, Negative-QTOF (Annotated)splash10-0a4i-9100000000-0d72fcffff267d7bc9802012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid Quattro_QQQ 40V, Negative-QTOF (Annotated)splash10-0zfs-9400000000-b3b27a9cc8fd4ebe9eb12012-07-24HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 10V, Positive-QTOFsplash10-052r-9500000000-543c65dad21c233fc8a82016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 20V, Positive-QTOFsplash10-052o-9000000000-7b7dcb34e28cb55ddafb2016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 40V, Positive-QTOFsplash10-0006-9000000000-27deee4c6a386a43ebc32016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 10V, Negative-QTOFsplash10-0udi-3900000000-a7aa709e1985355b4bd92016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 20V, Negative-QTOFsplash10-0pi0-9200000000-01a40fbe4d19283ef0802016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 40V, Negative-QTOFsplash10-0a4l-9000000000-622de763b5ea31ff4ee62016-09-12Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 10V, Positive-QTOFsplash10-0ap3-9000000000-6c76b805d02ad21ab06a2021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 20V, Positive-QTOFsplash10-0aou-9000000000-fc5e7dbe1e224cc87d4f2021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 40V, Positive-QTOFsplash10-0006-9000000000-021ab3207a43699617f42021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 10V, Negative-QTOFsplash10-0uki-9700000000-04713faa703ce789efb32021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 20V, Negative-QTOFsplash10-0a4r-9100000000-b0202d15bd6411bc7d802021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - (S)-3-Hydroxyisobutyric acid 40V, Negative-QTOFsplash10-052f-9000000000-d2c32880fd340aac0bad2021-09-25Wishart 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)2012-12-04Wishart 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
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified21.0 +/- 2.0 uMAdult (>18 years old)Both
Normal
details
BloodDetected and Quantified20.0 (4.0-48.0) uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified18.0 +/- 18.0 (0 - 36) uMAdult (>18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
SalivaDetected and Quantified1.23 +/- 0.71 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
details
UrineDetected and Quantified29.0 (11.8-59.8) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified10-17.0 umol/mmol creatinineInfant (0-1 year old)Male
Normal
details
UrineDetected and Quantified10.0-15.9 umol/mmol creatinineInfant (0-1 year old)Female
Normal
details
UrineDetected and Quantified6.9-13.4 umol/mmol creatinineInfant (0-1 year old)Female
Normal
details
UrineDetected and Quantified8.1-18 umol/mmol creatinineInfant (0-1 year old)Male
Normal
details
UrineDetected and Quantified13.39 +/- 6.588 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
details
UrineDetected and Quantified13.0 (2.0-24.0) umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified11 (4.1-19) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified<95 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
details
UrineDetected and Quantified14.5 (5.5-28.7) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified8.5 (4.9-17.1) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified7.8 (3.8-25.1) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified25.4 (10.2 -33.5) umol/mmol creatinineInfant (0-1 year old)BothNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified38.0 +/- 5.0 uMAdult (>18 years old)Both
Diabetes
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified12.772 +/- 10.179 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
UrineDetected and Quantified17.5 (2.0-33.0) umol/mmol creatinineAdult (>18 years old)Both3-Hydroxyisobutyric aciduria
    • MetaGene: Metabol...
details
UrineDetected and Quantified225.0 (60.0-390.0) umol/mmol creatinineChildren (1-13 years old)Both3-Hydroxyisobutyric Aciduria
    • MetaGene: Metabol...
details
Associated Disorders and Diseases
Disease References
Diabetes mellitus type 2
  1. Avogaro A, Bier DM: Contribution of 3-hydroxyisobutyrate to the measurement of 3-hydroxybutyrate in human plasma: comparison of enzymatic and gas-liquid chromatography-mass spectrometry assays in normal and in diabetic subjects. J Lipid Res. 1989 Nov;30(11):1811-7. [PubMed:2614280 ]
Colorectal cancer
  1. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  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 ]
3-Hydroxyisobutyric aciduria
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB021877
KNApSAcK IDNot Available
Chemspider ID389707
KEGG Compound IDC06001
BioCyc IDCPD-12175
BiGG ID37034
Wikipedia LinkNot Available
METLIN ID483
PubChem Compound440873
PDB IDNot Available
ChEBI ID37373
Food Biomarker OntologyNot Available
VMH ID3HMP
MarkerDB IDMDB00000012
Good Scents IDNot Available
References
Synthesis ReferenceRobison, Robert S.; Doremus, Michael G.L-(+)-b-Hydroxyisobutyric acid by fermentation. U.S. (1986), 5 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Yoshida I, Sweetman L, Kulovich S, Nyhan WL, Robinson BH: Effect of lipoic acid in a patient with defective activity of pyruvate dehydrogenase, 2-oxoglutarate dehydrogenase, and branched-chain keto acid dehydrogenase. Pediatr Res. 1990 Jan;27(1):75-9. [PubMed:2104971 ]
  2. Liebich HM, Dotzauer A, Tetschner B: Gas chromatographic determination of oxo- and hydroxycarboxylic acids in serum and urine of diabetic and normal subjects. J Chromatogr. 1989 May 12;468:157-65. [PubMed:2732286 ]
  3. Podebrad F, Heil M, Beck T, Mosandl A, Sewell AC, Bohles H: Stereodifferentiation of 3-hydroxyisobutyric- and 3-aminoisobutyric acid in human urine by enantioselective multidimensional capillary gas chromatography-mass spectrometry. Clin Chim Acta. 2000 Feb 25;292(1-2):93-105. [PubMed:10686279 ]
  4. Chitayat D, Meagher-Villemure K, Mamer OA, O'Gorman A, Hoar DI, Silver K, Scriver CR: Brain dysgenesis and congenital intracerebral calcification associated with 3-hydroxyisobutyric aciduria. J Pediatr. 1992 Jul;121(1):86-9. [PubMed:1625099 ]
  5. 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 ]
  6. Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed:8412012 ]
  7. Djoumbou-Feunang Y, Fiamoncini J, Gil-de-la-Fuente A, Greiner R, Manach C, Wishart DS: BioTransformer: a comprehensive computational tool for small molecule metabolism prediction and metabolite identification. J Cheminform. 2019 Jan 5;11(1):2. doi: 10.1186/s13321-018-0324-5. [PubMed:30612223 ]
  8. Viegas CM, da Costa Ferreira G, Schuck PF, Tonin AM, Zanatta A, de Souza Wyse AT, Dutra-Filho CS, Wannmacher CM, Wajner M: Evidence that 3-hydroxyisobutyric acid inhibits key enzymes of energy metabolism in cerebral cortex of young rats. Int J Dev Neurosci. 2008 May-Jun;26(3-4):293-9. doi: 10.1016/j.ijdevneu.2008.01.007. Epub 2008 Feb 7. [PubMed:18329219 ]

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
(S)-3-Hydroxyisobutyric acid → 2-methyl-3-(sulfooxy)propanoic aciddetails
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
EHHADH
Uniprot ID:
Q08426
Molecular weight:
69153.26
Reactions
(S)-3-Hydroxyisobutyric acid + NAD → (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Iondetails
General function:
Involved in 3-hydroxyacyl-CoA dehydrogenase activity
Specific function:
Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
Gene Name:
HADH
Uniprot ID:
Q16836
Molecular weight:
36035.11
Reactions
(S)-3-Hydroxyisobutyric acid + NAD → (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Iondetails
General function:
Involved in 3-hydroxyisobutyrate dehydrogenase activity
Specific function:
Not Available
Gene Name:
HIBADH
Uniprot ID:
P31937
Molecular weight:
35328.515
Reactions
(S)-3-Hydroxyisobutyric acid + NAD → 2-Methyl-3-oxopropanoic acid + NADHdetails
(S)-3-Hydroxyisobutyric acid + NAD → (S)-Methylmalonic acid semialdehyde + NADH + Hydrogen Iondetails
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
(S)-3-Hydroxyisobutyric acid → 6-(2-carboxy-2-methylethoxy)-3,4,5-trihydroxyoxane-2-carboxylic aciddetails
(S)-3-Hydroxyisobutyric acid → 3,4,5-trihydroxy-6-[(3-hydroxy-2-methylpropanoyl)oxy]oxane-2-carboxylic aciddetails
General function:
Involved in catalytic activity
Specific function:
Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
Gene Name:
HIBCH
Uniprot ID:
Q6NVY1
Molecular weight:
43481.935
Reactions
(S)-3-Hydroxyisobutyryl-CoA + Water → Coenzyme A + (S)-3-Hydroxyisobutyric aciddetails
(S)-3-Hydroxyisobutyryl-CoA + Water → Coenzyme A + (S)-3-Hydroxyisobutyric aciddetails