You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2008-08-13 17:24:17 UTC
Update Date2017-12-20 20:29:24 UTC
HMDB IDHMDB0006870
Secondary Accession Numbers
  • HMDB06870
Metabolite Identification
Common Name3-Hydroxyisovaleryl-CoA
Description3-hydroxyisovaleryl-coa, also known as 3-hydroxy-3-methylbutanoyl-coa, is a member of the class of compounds known as (s)-3-hydroxyacyl coas (s)-3-hydroxyacyl coas are organic compounds containing a (S)-3-hydroxyl acylated coenzyme A derivative. 3-hydroxyisovaleryl-coa is slightly soluble (in water) and an extremely strong acidic compound (based on its pKa). Within the cell, 3-hydroxyisovaleryl-coa is primarily located in the membrane (predicted from logP). It can also be found in the extracellular space. 3-hydroxyisovaleryl-coa exists in all living organisms, ranging from bacteria to humans. In humans, 3-hydroxyisovaleryl-coa is involved in the valine, leucine and isoleucine degradation. 3-hydroxyisovaleryl-coa is also involved in several metabolic disorders, some of which include isobutyryl-coa dehydrogenase deficiency, beta-ketothiolase deficiency, propionic acidemia, and 3-hydroxyisobutyric acid dehydrogenase deficiency. It is converted from 3-methylbut-2-enoyl-CoA by the enzyme enoyl-CoA hydratase..
Structure
Thumb
Synonyms
ValueSource
3-Hydroxy-3-methylbutanoyl-CoAHMDB
3-Hydroxy-3-methylbutanoyl-coenzyme AHMDB
3-Hydroxy-3-methylbutyryl-CoAHMDB
3-Hydroxy-3-methylbutyryl-coenzyme AHMDB
3-Hydroxyisovaleryl coenzyme AHMDB
Chemical FormulaC26H44N7O18P3S
Average Molecular Weight867.65
Monoisotopic Molecular Weight867.167637865
IUPAC Name{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-3-methylbutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid
Traditional Name[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-3-methylbutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid
CAS Registry NumberNot Available
SMILES
CC(C)(O)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N
InChI Identifier
InChI=1S/C26H44N7O18P3S/c1-25(2,20(37)23(38)29-6-5-15(34)28-7-8-55-16(35)9-26(3,4)39)11-48-54(45,46)51-53(43,44)47-10-14-19(50-52(40,41)42)18(36)24(49-14)33-13-32-17-21(27)30-12-31-22(17)33/h12-14,18-20,24,36-37,39H,5-11H2,1-4H3,(H,28,34)(H,29,38)(H,43,44)(H,45,46)(H2,27,30,31)(H2,40,41,42)/t14-,18-,19-,20?,24-/m1/s1
InChI KeyPEVZKILCBDEOBT-XBTRWLRFSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as (s)-3-hydroxyacyl coas. These are organic compounds containing a (S)-3-hydroxyl acylated coenzyme A derivative.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acyl thioesters
Direct Parent(S)-3-hydroxyacyl CoAs
Alternative Parents
Substituents
  • Coenzyme a or derivatives
  • Purine ribonucleoside 3',5'-bisphosphate
  • Purine ribonucleoside bisphosphate
  • Purine ribonucleoside diphosphate
  • Ribonucleoside 3'-phosphate
  • Pentose phosphate
  • Pentose-5-phosphate
  • Beta amino acid or derivatives
  • Glycosyl compound
  • N-glycosyl compound
  • 6-aminopurine
  • Monosaccharide phosphate
  • Organic pyrophosphate
  • Pentose monosaccharide
  • Imidazopyrimidine
  • Purine
  • Aminopyrimidine
  • Monoalkyl phosphate
  • N-acyl-amine
  • N-substituted imidazole
  • Organic phosphoric acid derivative
  • Monosaccharide
  • Imidolactam
  • Fatty amide
  • Phosphoric acid ester
  • Alkyl phosphate
  • Pyrimidine
  • Imidazole
  • Azole
  • Tetrahydrofuran
  • Heteroaromatic compound
  • Tertiary alcohol
  • Amino acid or derivatives
  • Thiocarboxylic acid ester
  • Carboxamide group
  • Carbothioic s-ester
  • Secondary alcohol
  • Secondary carboxylic acid amide
  • Sulfenyl compound
  • Thiocarboxylic acid or derivatives
  • Organoheterocyclic compound
  • Azacycle
  • Oxacycle
  • Carboxylic acid derivative
  • Organosulfur compound
  • Organic oxygen compound
  • Hydrocarbon derivative
  • Carbonyl group
  • Organic nitrogen compound
  • Primary amine
  • Organopnictogen compound
  • Organic oxide
  • Organooxygen compound
  • Organonitrogen compound
  • Alcohol
  • Amine
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External DescriptorsNot Available
Ontology
Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

    Cellular process:

Disposition

Biological location:

  Cell and elements:

  Subcellular:

Source:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility4.22 g/LALOGPS
logP-0.43ALOGPS
logP-6.9ChemAxon
logS-2.3ALOGPS
pKa (Strongest Acidic)0.83ChemAxon
pKa (Strongest Basic)4.95ChemAxon
Physiological Charge-4ChemAxon
Hydrogen Acceptor Count18ChemAxon
Hydrogen Donor Count10ChemAxon
Polar Surface Area383.86 ŲChemAxon
Rotatable Bond Count22ChemAxon
Refractivity187.67 m³·mol⁻¹ChemAxon
Polarizability78.09 ųChemAxon
Number of Rings3ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000i-1912000140-b0e9381d20810646f155View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-0913000000-4b1e16d242cdcfa4bea8View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-000i-2911000000-c2072cee5dd507dcde03View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-069s-9711140470-b93231a78f09a24923fcView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-003r-4910110010-b0a59a59576405a54451View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-057i-6900100000-f40a45ead54c1dc4f054View in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
Biofluid LocationsNot Available
Tissue LocationNot Available
Pathways
NameSMPDB/PathwhizKEGG
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencyPw000061Pw000061 greyscalePw000061 simpleNot Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencyPw000063Pw000063 greyscalePw000063 simpleNot Available
3-Methylcrotonyl Coa Carboxylase Deficiency Type IPw000065Pw000065 greyscalePw000065 simpleNot Available
3-Methylglutaconic Aciduria Type IPw000066Pw000066 greyscalePw000066 simpleNot Available
3-Methylglutaconic Aciduria Type IIIPw000067Pw000067 greyscalePw000067 simpleNot Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB024126
KNApSAcK IDNot Available
Chemspider ID10128175
KEGG Compound IDC05998
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound11953876
PDB IDNot Available
ChEBI ID28291
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General ReferencesNot Available

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
EHHADH
Uniprot ID:
Q08426
Molecular weight:
69153.26
Reactions
3-Hydroxyisovaleryl-CoA → 3-Methylcrotonyl-CoA + Waterdetails
General function:
Involved in catalytic activity
Specific function:
Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate.
Gene Name:
ECHS1
Uniprot ID:
P30084
Molecular weight:
31387.085
Reactions
3-Hydroxyisovaleryl-CoA → 3-Methylcrotonyl-CoA + Waterdetails
General function:
Involved in oxidoreductase activity
Specific function:
Bifunctional subunit.
Gene Name:
HADHA
Uniprot ID:
P40939
Molecular weight:
82998.97
Reactions
3-Hydroxyisovaleryl-CoA → 3-Methylcrotonyl-CoA + Waterdetails