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Showing metabocard for 2-Methyl-3-hydroxybutyryl-CoA (HMDB0001356)

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IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesenzymes (6) Show 6 proteinsXML
enzymes (6) Show 6 proteins
Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-02-26 21:23:15 UTC
HMDB IDHMDB0001356
Secondary Accession Numbers
  • HMDB0002029
  • HMDB01356
  • HMDB02029
Metabolite Identification
Common Name2-Methyl-3-hydroxybutyryl-CoA
Description2-Methyl-3-hydroxybutyryl-CoA, also known as (S)-3-hydroxy-2-methylbutyryl-CoA, 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. 2-Methyl-3-hydroxybutyryl-CoA is a strong basic compound (based on its pKa). 2-Methyl-3-hydroxybutyryl-CoA is a potentially toxic compound.
Structure
Data?1582752195
MOLSDFPDBSMILESInChI
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 KeyPEKYNTFSOBAABV-SYASONGASA-N
Chemical Taxonomy
Description 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
  • Monoalkyl phosphate
  • Aminopyrimidine
  • Imidolactam
  • N-acyl-amine
  • N-substituted imidazole
  • Organic phosphoric acid derivative
  • Monosaccharide
  • Pyrimidine
  • Alkyl phosphate
  • Fatty amide
  • Phosphoric acid ester
  • Tetrahydrofuran
  • Imidazole
  • Azole
  • Heteroaromatic compound
  • Carbothioic s-ester
  • Secondary alcohol
  • Thiocarboxylic acid ester
  • Carboxamide group
  • Secondary carboxylic acid amide
  • Amino acid or derivatives
  • 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
Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Industrial application:

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 g/LALOGPS
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 ŲChemAxon
Rotatable Bond Count22ChemAxon
Refractivity187.6 m³·mol⁻¹ChemAxon
Polarizability77.15 ųChemAxon
Number of Rings3ChemAxon
BioavailabilityNoChemAxon
Rule of FiveNoChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000i-0931000120-104f0c9e43205cba2cb5Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-002r-0911000000-854618a7f45296715eb3Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-002r-1910000000-05d47d13b00ed9efd2dbSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-00nb-9710041660-e7b1d428211e6f50d0a0Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-001i-4910220010-47f79cd44849bb97000cSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-056r-7900100000-b98220987a850d2126d4Spectrum
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Mitochondria
Biospecimen LocationsNot Available
Tissue LocationsNot Available
Pathways
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 IDFDB022575
KNApSAcK IDNot Available
Chemspider ID389295
KEGG Compound IDC04405
BioCyc IDNot Available
BiGG ID43751
Wikipedia LinkNot Available
METLIN ID6186
PubChem Compound440326
PDB IDNot Available
ChEBI ID15449
Food Biomarker OntologyNot Available
VMH IDNot Available
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. [PubMed:12872843 ]

Enzymes

Enzyme Details
1. 3-hydroxyacyl-CoA dehydrogenase type-2
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
Enzyme Details
2. Peroxisomal bifunctional enzyme
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
Enzyme Details
3. Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
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
Enzyme Details
4. Enoyl-CoA hydratase, mitochondrial
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
Enzyme Details
5. Trifunctional enzyme subunit alpha, mitochondrial
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
Enzyme Details
6. Peroxisomal multifunctional enzyme type 2
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