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Record Information
Version5.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-14 15:37:14 UTC
HMDB IDHMDB0001052
Secondary Accession Numbers
  • HMDB01052
Metabolite Identification
Common Name(S)-3-Hydroxyisobutyryl-CoA
Description(S)-3-Hydroxyisobutyryl-CoA is s metabolite of 3-hydroxyisobutyryl-CoA hydrolase (EC 3.1.2.4 ) during beta-alanine metabolism (KEGG 00410), propanoate metabolism (KEGG 00640), and valine, leucine and isoleucine degradation (KEGG 00280). Deficiencies of this enzyme in valine degradation can result in hypotonia, poor feeding, motor delay, and subsequent neurological regression in infancy, episodes of ketoacidosis and Leigh-like changes in the basal ganglia on a magnetic resonance imaging scan (PMID 17160907 ).
Structure
Data?1582752173
Synonyms
ValueSource
(S)-3-Hydroxyisobutyryl-coenzyme AHMDB
3-Hydroxy-2-methylpropanoyl-CoAHMDB
3-Hydroxy-2-methylpropanoyl-coenzyme AHMDB
3-Hydroxy-2-methylpropionyl-CoAHMDB
3-Hydroxy-2-methylpropionyl-coenzyme AHMDB
3-Hydroxy-isobutyryl-CoAHMDB
3-Hydroxy-isobutyryl-coenzyme AHMDB
3-Hydroxyisobutyryl-CoAHMDB
3-Hydroxyisobutyryl-coenzyme AHMDB
Chemical FormulaC25H42N7O18P3S
Average Molecular Weight853.623
Monoisotopic Molecular Weight853.151987801
IUPAC Name{[5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({[hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-2-methylpropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid
Traditional Name[5-(6-aminopurin-9-yl)-4-hydroxy-2-({[hydroxy([hydroxy(3-hydroxy-3-{[2-({2-[(3-hydroxy-2-methylpropanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}-2,2-dimethylpropoxy)phosphoryl]oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxyphosphonic acid
CAS Registry Number319440-43-2
SMILES
CC(CO)C(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OCC1OC(C(O)C1OP(O)(O)=O)N1C=NC2=C(N)N=CN=C12
InChI Identifier
InChI=1S/C25H42N7O18P3S/c1-13(8-33)24(38)54-7-6-27-15(34)4-5-28-22(37)19(36)25(2,3)10-47-53(44,45)50-52(42,43)46-9-14-18(49-51(39,40)41)17(35)23(48-14)32-12-31-16-20(26)29-11-30-21(16)32/h11-14,17-19,23,33,35-36H,4-10H2,1-3H3,(H,27,34)(H,28,37)(H,42,43)(H,44,45)(H2,26,29,30)(H2,39,40,41)
InChI KeyWWEOGFZEFHPUAM-UHFFFAOYSA-N
Chemical Taxonomy
Description 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 bisphosphate
  • Purine ribonucleoside 3',5'-bisphosphate
  • Ribonucleoside 3'-phosphate
  • Pentose-5-phosphate
  • Pentose phosphate
  • Beta amino acid or derivatives
  • Glycosyl compound
  • N-glycosyl compound
  • 6-aminopurine
  • Monosaccharide phosphate
  • Organic pyrophosphate
  • Imidazopyrimidine
  • Purine
  • Monoalkyl phosphate
  • Aminopyrimidine
  • Fatty amide
  • Monosaccharide
  • N-acyl-amine
  • N-substituted imidazole
  • Organic phosphoric acid derivative
  • Imidolactam
  • Phosphoric acid ester
  • Alkyl phosphate
  • Pyrimidine
  • Tetrahydrofuran
  • Azole
  • Heteroaromatic compound
  • Imidazole
  • Carbothioic s-ester
  • Secondary carboxylic acid amide
  • Secondary alcohol
  • Thiocarboxylic acid ester
  • Carboxamide group
  • Amino acid or derivatives
  • Thiocarboxylic acid or derivatives
  • Sulfenyl compound
  • Carboxylic acid derivative
  • Organoheterocyclic compound
  • Oxacycle
  • Azacycle
  • Hydrocarbon derivative
  • Organic nitrogen compound
  • Organic oxide
  • Organosulfur compound
  • Organonitrogen compound
  • Primary amine
  • Organopnictogen compound
  • Carbonyl group
  • Organic oxygen compound
  • Primary alcohol
  • Organooxygen 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 Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility4.14 g/LALOGPS
logP-0.56ALOGPS
logP-5.9ChemAxon
logS-2.3ALOGPS
pKa (Strongest Acidic)0.82ChemAxon
pKa (Strongest Basic)4.01ChemAxon
Physiological Charge-4ChemAxon
Hydrogen Acceptor Count18ChemAxon
Hydrogen Donor Count10ChemAxon
Polar Surface Area383.86 ŲChemAxon
Rotatable Bond Count22ChemAxon
Refractivity183.18 m³·mol⁻¹ChemAxon
Polarizability75.7 ųChemAxon
Number of Rings3ChemAxon
BioavailabilityNoChemAxon
Rule of FiveNoChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleYesChemAxon
Predicted Spectral Properties

Collision Cross Sections

NameAdductTypeData SourceValueReference
DarkChem[M+H]+PredictedNot Available285.36331661259
DarkChem[M-H]-PredictedNot Available264.68831661259
AllCCS[M+H]+PredictedNot Available257.09732859911
AllCCS[M-H]-PredictedNot Available255.44532859911

Retention Indices

Underivatized

Not Available

Derivatized

Not Available
Spectra

LC-MS/MS

Spectrum TypeDescriptionSplash KeyDeposition DateView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000i-1912000120-c718f8aabcd5b33281932015-09-14View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-1913000000-86eb8b4e6cdee60b559a2015-09-14View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-000i-2911000000-2d6f48b3506835c89ffd2015-09-14View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-00o9-9730140560-056ac34ed9b661751c572015-09-15View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-001i-3920110010-37a68eba22e3eecc97572015-09-15View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-057i-6900100000-e33bac5e5de19c7b719b2015-09-15View Spectrum
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
FooDB IDFDB022393
KNApSAcK IDNot Available
Chemspider ID86
KEGG Compound IDC06000
BioCyc IDNot Available
BiGG ID47176
Wikipedia LinkNot Available
METLIN ID453
PubChem Compound88
PDB IDNot Available
ChEBI ID28259
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
References
Synthesis ReferenceHawes, John W.; Harper, Edwin T. Synthesis of methacrylyl-CoA and (R)- and (S)-3-hydroxyisobutyryl-CoA. Methods in Enzymology (2000), 324(Branched-Chain Amino Acids), 73-79.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Lehnert W, Sass JO: Glutaconyl-CoA is the main toxic agent in glutaryl-CoA dehydrogenase deficiency (glutaric aciduria type I). Med Hypotheses. 2005;65(2):330-3. [PubMed:15922108 ]
  2. Loupatty FJ, Clayton PT, Ruiter JP, Ofman R, Ijlst L, Brown GK, Thorburn DR, Harris RA, Duran M, Desousa C, Krywawych S, Heales SJ, Wanders RJ: Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration. Am J Hum Genet. 2007 Jan;80(1):195-9. Epub 2006 Nov 30. [PubMed:17160907 ]

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
EHHADH
Uniprot ID:
Q08426
Molecular weight:
69153.26
Reactions
Methacrylyl-CoA + Water → (S)-3-Hydroxyisobutyryl-CoAdetails
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
Methacrylyl-CoA + Water → (S)-3-Hydroxyisobutyryl-CoAdetails
General function:
Involved in oxidoreductase activity
Specific function:
Bifunctional subunit.
Gene Name:
HADHA
Uniprot ID:
P40939
Molecular weight:
82998.97
Reactions
Methacrylyl-CoA + Water → (S)-3-Hydroxyisobutyryl-CoAdetails
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