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Bovine Metabolome Database



Showing metabocard for (S)-3-Hydroxyisobutyryl-CoA (BMDB01052)

Legend: metabolite field enzyme field

Version 1.0
Creation Date 2005-11-16 15:48:42
Update Date 2009-05-05 20:58:27
Accession Number BMDB01052
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).
Synonyms
  1. 3-Hydroxy-2-methylpropanoyl-CoA
  2. 3-Hydroxy-2-methylpropionyl-CoA
  3. 3-hydroxy-isobutyryl-CoA
  4. 3-hydroxy-isobutyryl-coenzyme A
  5. (S)-3-Hydroxyisobutyryl-CoA
  6. 3-Hydroxy-2-methylpropanoyl-Coenzyme A
  7. 3-Hydroxy-2-methylpropionyl-Coenzyme A
  8. (S)-3-Hydroxyisobutyryl-Coenzyme A
  9. 3-hydroxyisobutyryl-CoA
  10. 3-hydroxyisobutyryl-Coenzyme A
Chemical IUPAC Name S-[2-[3-[[4-[[[5-(6-aminopurin-9-yl)-4-hydroxy-3-phosphonooxyoxolan-2-yl]methoxy-hydroxyphosphoryl]oxy-hydroxyphosphoryl]oxy-2-hydroxy-3,3-dimethylbutanoyl]amino]propanoylamino]ethyl] 3-hydroxy-2-methylpropanethioate
Chemical Formula C25H42N7O18P3S
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Nucleosides and Nucleoside conjugates
Class
  • Coenzyme A Derivatives
Sub Class
  • Short chain acyl CoAs
Family
  • Mammalian_Metabolite
Species
  • primary alcohol; secondary alcohol; primary amine; primary aromatic amine; secondary carboxylic acid amide; thiocarboxylic acid ester; phosphoric acid ester; aromatic compound; heterocyclic compound
Biofunction
  • Lipid biosynthesis, Fatty acid transport
Application
Source
  • Endogenous
Average Molecular Weight 853.623
Monoisotopic Molecular Weight 853.151978
Isomeric 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
Canonical 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
KEGG Compound ID C06000 Link Image
BioCyc ID 3-HYDROXY-ISOBUTYRYL-COA Link Image
BiGG ID 47176 Link Image
Wikipedia Link Not Available
METLIN ID 453 Link Image
PubChem Compound 88 Link Image
PubChem Substance 8274 Link Image
ChEBI ID 28259 Link Image
CAS Registry Number 319440-43-2
InChI Identifier InChI=1/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)
Synthesis Reference Hawes, 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.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 4.14 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -4
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity -6.7 [Predicted by PubChem via XLOGP]; -0.56 [Predicted by ALOGPS] Calculated using ALOGPS
Material Safety Data Sheet (MSDS) Not Available
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Not Available
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Not Available
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
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Mass Spectrum Not Available
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm (Predicted from LogP)
  • mitochondria
Biofluid Location Not Available
Tissue Location Not Available
Concentrations (Normal) Not Available
Concentrations (Abnormal) Not Available
Pathway Names
  • Valine, Leucine and Isoleucine Degradation
HMDB Pathways
Name Valine, Leucine and Isoleucine Degradation
Image Show Link Image
KEGG Pathways
Name Valine, Leucine and Isoleucine Degradation
Image Show Link Image
SimCell Pathways
Name Valine, Leucine and Isoleucine Degradation
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Graph Show Link Image
SBML Download (XML) Link Image
General References Not Available
Metabolic Enzymes
  1. 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
Enzyme 1 [top]
Enzyme 1 ID 549
Enzyme 1 Name 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
Enzyme 1 Synonyms
  1. 3-hydroxyisobutyryl-coenzyme A hydrolase
  2. HIB-CoA hydrolase
  3. HIBYL-CoA-H
Enzyme 1 Gene Name HIBCH
Enzyme 1 Protein Sequence >3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
MGLQGLCRLMSRFNSYKRTNIILQHLKMSNHTDAAAEVLLERKGCAGVITLNRPRFLNTL
TLGMIRQIYAQLKKWEQDPKTFLIIIKGAGEKAFCAGGDIRALSEARNTNQKMLQDLFRE
EYILNNAIDSCQKPYIALIHGITMGGGVGVSVHGQFRVATEKSVFAMPETAIGLFPDVGG
GYFLPRLQGKLGYFLALTGFRLKGRDVYTAGIATHFVDFEKLGMLEEDLLALKSPSKENI
ADVLETYHAKSKTDQDKPFILEEHMDKINSWFSANTVEQIVDNLQQDGSSFALEQLKVIK
KMSPTSLKITLRQLMEGSSKTLPEVLIMEYRLSQACMKGHDFHEGVRAVLIDKDQSPKWK
PADLKEVTDEDLNDYFKSLGSNDLKF
Enzyme 1 Number of Residues 386
Enzyme 1 Molecular Weight 43348.7
Enzyme 1 Theoretical pI 8.21
Enzyme 1 GO Classification
Function
Process
Component
Enzyme 1 General Function Lipid transport and metabolism
Enzyme 1 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
Enzyme 1 Pathways
  • Amino-acid degradation
  • L-valine degradation
Enzyme 1 Reactions Not Available
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 87578354 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID Q2HJ73 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name HIBCH_BOVIN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1161 bp
ATGGGGTTGCAGGGGTTGTGCAGACTCATGTCGAGGTTTAATTCATATAAAAGGACTAAT
ATCATACTGCAGCATTTGAAAATGTCTAATCACACAGATGCAGCAGCAGAAGTCCTGTTG
GAAAGAAAAGGTTGTGCAGGAGTGATAACACTAAACAGACCAAGGTTTCTGAATACCCTG
ACTCTTGGTATGATTCGGCAGATTTACGCACAGCTAAAGAAGTGGGAACAAGATCCGAAA
ACTTTCCTGATCATCATAAAGGGAGCTGGTGAAAAGGCTTTCTGTGCTGGAGGTGATATC
AGAGCACTCTCAGAAGCTAGAAATACAAACCAGAAAATGCTTCAAGATTTATTCCGAGAG
GAATATATTCTGAACAATGCCATTGATTCTTGCCAGAAACCATATATTGCACTTATTCAT
GGAATTACAATGGGTGGGGGAGTTGGTGTCTCAGTTCATGGGCAATTCCGAGTGGCTACC
GAAAAGTCTGTTTTTGCGATGCCAGAAACAGCAATAGGGCTATTCCCTGATGTGGGTGGA
GGTTATTTCTTGCCACGACTTCAAGGAAAACTTGGGTACTTCCTTGCATTAACAGGATTC
AGGCTGAAAGGAAGAGATGTGTACACAGCAGGAATTGCTACACACTTCGTAGATTTTGAA
AAGTTGGGCATGCTAGAAGAAGATTTGTTAGCCCTGAAATCTCCTTCAAAAGAAAATATT
GCAGATGTCTTAGAAACTTACCATGCAAAGTCAAAGACTGATCAAGACAAGCCTTTTATA
CTCGAGGAACACATGGACAAAATAAACAGTTGGTTCTCAGCTAATACTGTGGAACAGATT
GTTGACAATTTACAGCAAGACGGCTCATCTTTTGCCCTGGAGCAGCTGAAGGTAATTAAA
AAAATGTCTCCAACTTCACTAAAGATCACACTAAGGCAACTCATGGAGGGGTCTTCAAAG
ACCTTGCCAGAAGTATTAATTATGGAATATCGGCTGAGTCAAGCTTGTATGAAAGGCCAT
GACTTTCATGAAGGCGTTAGGGCAGTTTTAATAGATAAAGACCAGAGTCCAAAGTGGAAA
CCAGCTGACCTAAAAGAAGTTACTGATGAAGATTTGAATGATTACTTCAAATCTCTGGGA
AGTAATGATTTGAAATTTTGA
Enzyme 1 GenBank Gene ID BC113274 Link Image
Enzyme 1 GeneCard ID HIBCH Link Image
Enzyme 1 GenAtlas ID Not Available
Enzyme 1 HGNC ID Not Available
Enzyme 1 Chromosome Location Chromosome:2
Enzyme 1 Locus 2q32.2
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References Not Available
Enzyme 1 Metabolite References Not Available