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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:04:24 UTC
HMDB IDHMDB01356
Secondary Accession Numbers
  • HMDB02029
Metabolite Identification
Common Name2-Methyl-3-hydroxybutyryl-CoA
Description2-Methyl-3-hydroxybutyryl-CoA is a substrate for 3-hydroxyacyl-CoA dehydrogenase type II, Enoyl-CoA hydratase (mitochondrial), Trifunctional enzyme alpha subunit (mitochondrial), Short chain 3-hydroxyacyl-CoA dehydrogenase (mitochondrial) and Peroxisomal bifunctional enzyme.
Structure
Thumb
Synonyms
ValueSource
(2S,3S)-3-Hydroxy-2-methylbutanoyl-CoAHMDB
(2S,3S)-3-Hydroxy-2-methylbutanoyl-coenzyme AHMDB
(2S,3S)-3-Hydroxy-2-methylbutyryl-CoAHMDB
(2S,3S)-3-Hydroxy-2-methylbutyryl-coenzyme AHMDB
(S)-3-Hydroxy-2-methylbutyryl-CoAHMDB
(S)-3-Hydroxy-2-methylbutyryl-coenzyme AHMDB
2-Methyl-3-hydroxybutyryl-coenzyme AHMDB
3-Hydroxy-2-methylbutyryl-CoAHMDB
3-Hydroxy-2-methylbutyryl-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-{[(2S,3S)-3-hydroxy-2-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-{[(2S,3S)-3-hydroxy-2-methylbutanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)-2,2-dimethylpropoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid
CAS Registry Number52227-66-4
SMILES
C[C@H](O)[C@H](C)C(=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/c1-13(14(2)34)25(39)55-8-7-28-16(35)5-6-29-23(38)20(37)26(3,4)10-48-54(45,46)51-53(43,44)47-9-15-19(50-52(40,41)42)18(36)24(49-15)33-12-32-17-21(27)30-11-31-22(17)33/h11-15,18-20,24,34,36-37H,5-10H2,1-4H3,(H,28,35)(H,29,38)(H,43,44)(H,45,46)(H2,27,30,31)(H2,40,41,42)/t13-,14-,15+,18+,19+,20?,24+/m0/s1
InChI KeyInChIKey=PEKYNTFSOBAABV-SYASONGASA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acyl thioesters
Direct ParentAcyl CoAs
Alternative Parents
Substituents
  • Coenzyme a or derivatives
  • Purine ribonucleoside diphosphate
  • Purine ribonucleoside 3',5'-bisphosphate
  • N-glycosyl compound
  • Glycosyl compound
  • Beta amino acid or derivatives
  • Organic pyrophosphate
  • Monosaccharide phosphate
  • 6-aminopurine
  • Purine
  • Imidazopyrimidine
  • Monoalkyl phosphate
  • Aminopyrimidine
  • Imidolactam
  • Alkyl phosphate
  • Pyrimidine
  • Primary aromatic amine
  • Phosphoric acid ester
  • Organic phosphoric acid derivative
  • Organic phosphate
  • N-substituted imidazole
  • N-acyl-amine
  • Monosaccharide
  • Fatty amide
  • Heteroaromatic compound
  • Oxolane
  • Imidazole
  • Azole
  • Thiocarboxylic acid ester
  • Secondary carboxylic acid amide
  • Secondary alcohol
  • Carboxamide group
  • Oxacycle
  • Azacycle
  • Organoheterocyclic compound
  • Sulfenyl compound
  • Thioether
  • Thiocarboxylic acid or derivatives
  • Carboxylic acid derivative
  • Carboxylic acid amide
  • Hydrocarbon derivative
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Alcohol
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External DescriptorsNot Available
Ontology
StatusExpected but not Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Lipid biosynthesis, Fatty acid transport
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
  • Mitochondria
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 Solubility3.96 mg/mLALOGPS
logP-0.48ALOGPS
logP-6.6ChemAxon
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 Å2ChemAxon
Rotatable Bond Count22ChemAxon
Refractivity187.6 m3·mol-1ChemAxon
Polarizability77.15 Å3ChemAxon
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, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, PositiveNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, NegativeNot Available
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, NegativeNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid LocationsNot Available
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencySMP00137Not Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencySMP00138Not Available
3-hydroxyisobutyric acid dehydrogenase deficiencySMP00521Not Available
3-hydroxyisobutyric aciduriaSMP00522Not Available
3-Methylcrotonyl Coa Carboxylase Deficiency Type ISMP00237Not Available
3-Methylglutaconic Aciduria Type ISMP00139Not Available
3-Methylglutaconic Aciduria Type IIISMP00140Not Available
3-Methylglutaconic Aciduria Type IVSMP00141Not Available
Beta-Ketothiolase DeficiencySMP00173Not Available
Isobutyryl-coa dehydrogenase deficiencySMP00523Not Available
Isovaleric acidemiaSMP00524Not Available
Isovaleric AciduriaSMP00238Not Available
Maple Syrup Urine DiseaseSMP00199Not Available
Methylmalonate Semialdehyde Dehydrogenase DeficiencySMP00384Not Available
Methylmalonic AciduriaSMP00200Not Available
Propionic AcidemiaSMP00236Not Available
Valine, Leucine and Isoleucine DegradationSMP00032map00280
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022575
KNApSAcK IDNot Available
Chemspider ID389295
KEGG Compound IDC04405
BioCyc ID2-METHYL-3-HYDROXY-BUTYRYL-COA
BiGG ID43751
Wikipedia LinkNot Available
NuGOwiki LinkHMDB01356
Metagene LinkHMDB01356
METLIN ID6186
PubChem Compound440326
PDB IDNot Available
ChEBI ID15449
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Sutton VR, O'Brien WE, Clark GD, Kim J, Wanders RJ: 3-Hydroxy-2-methylbutyryl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 2003;26(1):69-71. [12872843 ]

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD).
Gene Name:
HSD17B10
Uniprot ID:
Q99714
Molecular weight:
25983.695
Reactions
2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADHdetails
2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
EHHADH
Uniprot ID:
Q08426
Molecular weight:
69153.26
Reactions
2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH + Hydrogen Iondetails
2-Methyl-3-hydroxybutyryl-CoA → Tiglyl-CoA + Waterdetails
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
2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH + Hydrogen Iondetails
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
2-Methyl-3-hydroxybutyryl-CoA → Tiglyl-CoA + Waterdetails
General function:
Involved in oxidoreductase activity
Specific function:
Bifunctional subunit.
Gene Name:
HADHA
Uniprot ID:
P40939
Molecular weight:
82998.97
Reactions
2-Methyl-3-hydroxybutyryl-CoA → Tiglyl-CoA + Waterdetails
General function:
Involved in oxidoreductase activity
Specific function:
Bifunctional enzyme acting on the peroxisomal beta-oxidation pathway for fatty acids. Catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids.
Gene Name:
HSD17B4
Uniprot ID:
P51659
Molecular weight:
79685.715