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Bovine Metabolome Database



Showing metabocard for (S)-3-Hydroxyisobutyric acid (BMDB00023)

Legend: metabolite field enzyme field

Version 1.0
Creation Date 2005-11-16 15:48:42
Update Date 2009-07-29 11:39:31
Accession Number BMDB00023
Common Name (S)-3-Hydroxyisobutyric acid
Description (S)-3-Hydroxyisobutyric (3-HIBA) acid is an organic acid. 3-HIBA is an intermediate in the metabolic pathways of L-valine and L-thymine amino acids. 3-HIBA plays an important role in the diagnosis of the very rare inherited metabolic diseases 3-hydroxyisobutyric aciduria (OMIM 236795) and methylmalonic semialdehyde dehydrogenase deficiency (OMIM 603178). Patients with 3-hydroxyisobutyric aciduria excrete a significant amount of 3-HIBA not only during the acute stage but also when stable. The deficient enzyme in 3HiB-uria remains unclear. The severity of this disease varies from case to case. Most patients exhibit dysmorphic features, such as a small triangular face, a long philtrum, low set ears and micrognathia (PMID: 113770040, 10686279)
Synonyms
  1. (S)-3-Hydroxyisobutyric acid
  2. 2-methyl-L-(+)-Hydracrylate
  3. 2-methyl-L-(+)-Hydracrylic acid
  4. 3-Hydroxy-2-methylpropanoate
  5. 3-Hydroxy-2-methylpropanoic acid
  6. 3-Hydroxyisobutyrate
  7. 3-Hydroxyisobutyric acid
  8. 3-hydroxy-2-methyl-(S)-Propanoate
  9. 3-hydroxy-2-methyl-(S)-Propanoic acid
  10. 3-hydroxy-isobutyrate
Chemical IUPAC Name 3-hydroxy-2-methylpropanoic acid
Chemical Formula C4H8O3
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Organic acids
Class
  • Hydroxy Acids; Fatty Acids
Sub Class
  • Short chain hydroxy acids
Family
  • Mammalian_Metabolite
Species
  • primary alcohol; carboxylic acid
Biofunction
Application
Source
  • Endogenous
Average Molecular Weight 104.104
Monoisotopic Molecular Weight 104.047340
Isomeric SMILES C[C@@H](CO)C(O)=O
Canonical SMILES CC(CO)C(O)=O
KEGG Compound ID C01188 Link Image
BioCyc ID 3-HYDROXY-ISOBUTYRATE Link Image
BiGG ID 37034 Link Image
Wikipedia Link Not Available
METLIN ID 483 Link Image
PubChem Compound 87 Link Image
PubChem Substance 37904468 Link Image
ChEBI ID 18064 Link Image
CAS Registry Number 2068-83-9
InChI Identifier InChI=1/C4H8O3/c1-3(2-5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1
Synthesis Reference Robison, Robert S.; Doremus, Michael G.L-(+)-b-Hydroxyisobutyric acid by fermentation. U.S. (1986), 5 pp.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 571.0 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity -0.47 [Predicted by ALOGPS]; -0.7 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Download Spectrum
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Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Download Spectrum
Download FID (Bruker)
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Predicted 1H NMR Spectrum Show Image
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Predicted 13C NMR Spectrum Show Image
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Mass Spectrum
Low Energy
Download File
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Medium Energy
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High Energy
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Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm
  • 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
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SimCell Pathways
Name Valine, Leucine and Isoleucine Degradation
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SBML Download (XML) Link Image
General References Not Available
Metabolic Enzymes
  1. 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
  2. 3-hydroxyisobutyrate dehydrogenase, 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
Enzyme 2 [top]
Enzyme 2 ID 550
Enzyme 2 Name 3-hydroxyisobutyrate dehydrogenase, mitochondrial
Enzyme 2 Synonyms
  1. HIBADH
Enzyme 2 Gene Name HIBADH
Enzyme 2 Protein Sequence >3-hydroxyisobutyrate dehydrogenase, mitochondrial
MAASLRLRGAASGLRYWSRRQPPAVASLAAVCSRSMASKTPVGFIGVGNMGNPMAKNLMK
HGYPLIIYDVFPDACKEFLDAGEQVVSSPADVAEKADRIITMLPTSINAIEAYSGANGIL
KKVKKGSLLIDSSTIDPMVSKELAKEVEKMGAVFMDAPVSGGVGAARSGNLTFMVGGVEE
EFAAAQELLGCMGSNVVYCGAVGTGQAAKICNNLLLAISMIGTAEAMNLGIRLGLDPKLL
AKILNMSSGRCWSSDTYNPVPGVMDGVPSANNYQGGFGTTLMAKDLGLAQDSATSTKSPI
LLGSQAHQIYRMMCAKGYSKKDFSSVFQFLREEETF
Enzyme 2 Number of Residues 336
Enzyme 2 Molecular Weight 35409.7
Enzyme 2 Theoretical pI 8.26
Enzyme 2 GO Classification
Function
Process
Component
Enzyme 2 General Function Lipid transport and metabolism
Enzyme 2 Specific Function 3-hydroxy-2-methylpropanoate + NAD(+) = 2- methyl-3-oxopropanoate + NADH
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions Not Available
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 88682977 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID Q2HJD7 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name 3HIDH_BOVIN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence >1011 bp
ATGGCAGCCTCCTTACGGCTCCGTGGAGCCGCCTCCGGCCTCCGGTATTGGAGCCGCCGG
CAGCCTCCGGCAGTGGCCAGCCTTGCAGCGGTGTGTTCTAGGTCAATGGCTTCTAAGACT
CCAGTTGGATTCATTGGAGTTGGCAACATGGGGAATCCAATGGCAAAAAATCTCATGAAA
CATGGCTATCCACTTATTATTTATGATGTGTTCCCTGATGCTTGCAAAGAGTTTCTAGAT
GCAGGTGAACAGGTAGTGTCTTCCCCAGCAGATGTAGCTGAAAAAGCTGACAGAATTATT
ACCATGTTGCCCACCAGCATCAATGCGATAGAAGCTTATTCTGGAGCAAATGGAATTCTA
AAAAAAGTGAAGAAAGGCTCACTATTAATAGATTCCAGTACTATTGATCCTATGGTTTCA
AAAGAACTGGCCAAAGAAGTTGAAAAAATGGGAGCAGTTTTCATGGATGCCCCTGTTTCT
GGTGGTGTGGGAGCCGCTCGGTCGGGGAACCTCACATTTATGGTAGGAGGAGTCGAAGAA
GAATTTGCTGCTGCCCAAGAGTTGCTGGGGTGCATGGGCTCCAATGTGGTGTATTGTGGA
GCTGTTGGGACAGGGCAGGCTGCGAAGATCTGCAACAACTTGCTGTTAGCTATTAGTATG
ATTGGAACTGCTGAAGCTATGAATCTGGGAATCAGGTTAGGGCTTGACCCAAAACTCTTG
GCTAAAATCCTAAATATGAGCTCAGGACGATGTTGGTCAAGTGACACTTATAATCCTGTA
CCTGGGGTGATGGATGGAGTTCCCTCGGCTAATAACTATCAGGGCGGATTCGGAACAACA
CTCATGGCGAAGGATCTGGGATTAGCACAAGACTCTGCCACCAGCACGAAGAGCCCAATC
CTTCTGGGCAGTCAAGCCCATCAGATCTACAGGATGATGTGTGCAAAAGGCTACTCAAAA
AAAGACTTCTCATCCGTGTTCCAGTTTCTACGAGAGGAGGAGACATTCTGA
Enzyme 2 GenBank Gene ID BC105543 Link Image
Enzyme 2 GeneCard ID HIBADH Link Image
Enzyme 2 GenAtlas ID Not Available
Enzyme 2 HGNC ID Not Available
Enzyme 2 Chromosome Location Chromosome:7
Enzyme 2 Locus 7p15.2
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References Not Available
Enzyme 2 Metabolite References Not Available