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 |
- (S)-3-Hydroxyisobutyric acid
- 2-methyl-L-(+)-Hydracrylate
- 2-methyl-L-(+)-Hydracrylic acid
- 3-Hydroxy-2-methylpropanoate
- 3-Hydroxy-2-methylpropanoic acid
- 3-Hydroxyisobutyrate
- 3-Hydroxyisobutyric acid
- 3-hydroxy-2-methyl-(S)-Propanoate
- 3-hydroxy-2-methyl-(S)-Propanoic acid
- 3-hydroxy-isobutyrate
|
Chemical IUPAC Name |
3-hydroxy-2-methylpropanoic acid |
Chemical Formula |
C4H8O3 |
Chemical Structure |
 |
Chemical Taxonomy |
Kingdom |
|
Super Class |
|
Class |
- Hydroxy Acids; Fatty Acids
|
Sub Class |
- Short chain hydroxy acids
|
Family |
|
Species |
- primary alcohol; carboxylic acid
|
Biofunction |
— |
Application |
— |
Source |
|
|
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  |
BioCyc ID |
3-HYDROXY-ISOBUTYRATE  |
BiGG ID |
37034  |
Wikipedia Link |
Not Available |
METLIN ID |
483  |
PubChem Compound |
87  |
PubChem Substance |
37904468  |
ChEBI ID |
18064  |
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 Download FID (Varian) Show Experimental Conditions  |
Experimental 13C NMR Spectrum |
Not Available |
Experimental 13C HSQC Spectrum |
Download Spectrum Download FID (Bruker) Show Experimental Conditions  |
Predicted 1H NMR Spectrum |
Show Image Show Peaklist
|
Predicted 13C NMR Spectrum |
Show Image Show Peaklist
|
Mass Spectrum |
|
Simplified TOCSY Spectrum |
Not Available |
BMRB Spectrum |
Not Available |
Cellular Location |
|
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  |
|
KEGG Pathways |
Name
|
Valine, Leucine and Isoleucine Degradation
|
Image
|
Show  |
|
SimCell Pathways |
|
General References |
Not Available |
Metabolic Enzymes |
- 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
- 3-hydroxyisobutyrate dehydrogenase, mitochondrial
|
Enzyme 1
[top]
|
Enzyme 1 ID |
549 |
Enzyme 1 Name |
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial |
Enzyme 1 Synonyms |
- 3-hydroxyisobutyryl-coenzyme A hydrolase
- HIB-CoA hydrolase
- 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 |
|
Enzyme 1 Transmembrane Regions |
|
Enzyme 1 Essentiality |
Not Available |
Enzyme 1 GenBank ID Protein |
87578354  |
Enzyme 1 UniProtKB/Swiss-Prot ID |
Q2HJ73  |
Enzyme 1 UniProtKB/Swiss-Prot Entry Name |
HIBCH_BOVIN  |
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  |
Enzyme 1 GeneCard ID |
HIBCH  |
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  |
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 |
- 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 |
|
Enzyme 2 Transmembrane Regions |
|
Enzyme 2 Essentiality |
Not Available |
Enzyme 2 GenBank ID Protein |
88682977  |
Enzyme 2 UniProtKB/Swiss-Prot ID |
Q2HJD7  |
Enzyme 2 UniProtKB/Swiss-Prot Entry Name |
3HIDH_BOVIN  |
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  |
Enzyme 2 GeneCard ID |
HIBADH  |
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  |
Enzyme 2 General References |
Not Available |
Enzyme 2 Metabolite References |
Not Available |