| Identification |
|---|
| HMDB Protein ID
| CDBP02423 |
| Secondary Accession Numbers
| Not Available |
| Name
| 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial |
| Description
| Not Available |
| Synonyms
|
- 3-hydroxyisobutyryl-coenzyme A hydrolase
- HIB-CoA hydrolase
- HIBYL-CoA-H
|
| Gene Name
| HIBCH |
| Protein Type
| Enzyme |
| Biological Properties |
|---|
| 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.
|
| GO Classification
|
| Biological Process |
| branched-chain amino acid catabolic process |
| cellular nitrogen compound metabolic process |
| valine catabolic process |
| Cellular Component |
| mitochondrial matrix |
| Function |
| catalytic activity |
| Molecular Function |
| 3-hydroxyisobutyryl-CoA hydrolase activity |
| Process |
| metabolic process |
|
| Cellular Location
|
- Mitochondrion
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | Propanoate Metabolism |    |  | | Valine, Leucine and Isoleucine Degradation |    |  | | L-valine degradation | Not Available | Not Available | | Beta-Ketothiolase Deficiency |    | Not Available | | 2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency |    | Not Available |
|
| Gene Properties |
|---|
| Chromosome Location
| 2 |
| Locus
| 2q32.2 |
| SNPs
| HIBCH |
| Gene Sequence
|
>1161 bp
ATGGGGCAGCGCGAGATGTGGAGGCTCATGTCGAGGTTTAATGCATTCAAAAGGACTAAT
ACCATACTGCACCATTTGAGAATGTCCAAGCACACAGATGCAGCAGAAGAGGTGCTATTG
GAAAAAAAAGGTTGCACGGGAGTCATAACACTAAACAGACCAAAGTTCCTCAATGCACTG
ACTCTTAATATGATTCGGCAGATTTATCCACAGCTAAAGAAGTGGGAACAAGATCCTGAA
ACTTTCCTGATCATTATAAAGGGAGCAGGAGGAAAGGCTTTCTGTGCCGGGGGTGATATC
AGAGTGATCTCGGAAGCTGAAAAGGCAAAACAGAAGATAGCTCCAGTTTTCTTCAGAGAA
GAATATATGCTGAATAATGCTGTTGGTTCTTGCCAGAAACCTTATGTTGCACTTATTCAT
GGAATTACAATGGGTGGGGGAGTTGGTCTCTCAGTCCATGGGCAATTTCGAGTGGCTACA
GAAAAGTGTCTTTTTGCTATGCCAGAAACTGCAATAGGACTGTTCCCTGATGTGGGTGGA
GGTTATTTCTTGCCACGACTCCAAGGAAAACTTGGTTACTTCCTTGCATTAACAGGATTC
AGACTAAAAGGAAGAGATGTGTACAGAGCAGGAATTGCTACACACTTTGTAGATTCTGAA
AAGTTGGCCATGTTAGAGGAAGATTTGTTAGCCTTGAAATCTCCTTCAAAAGAAAATATT
GCATCTGTCTTAGAAAATTACCATACAGAGTCTAAGATTGATCGAGACAAGTCTTTTATA
CTTGAGGAACACATGGACAAAATAAACAGTTGTTTTTCAGCCAATACTGTGGAAGAAATT
ATTGAAAACTTACAGCAAGATGGTTCATCTTTTGCCCTAGAGCAATTGAAGGTAATTAAT
AAAATGTCTCCAACATCTCTAAAGATCACACTAAGGCAACTCATGGAGGGGTCTTCAAAG
ACCTTGCAAGAAGTACTAACTATGGAGTATCGGCTAAGTCAAGCTTGTATGAGAGGTCAT
GACTTTCATGAAGGCGTTAGAGCTGTTTTAATTGATAAAGACCAGAGTCCAAAATGGAAA
CCAGCTGATCTAAAAGAAGTTACTGAGGAAGATTTGAATAATCACTTTAAGTCTTTGGGA
AGCAGTGATTTGAAATTTTGA
|
| Protein Properties |
|---|
| Number of Residues
| 386 |
| Molecular Weight
| 43481.935 |
| Theoretical pI
| 8.196 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
MGQREMWRLMSRFNAFKRTNTILHHLRMSKHTDAAEEVLLEKKGCTGVITLNRPKFLNAL
TLNMIRQIYPQLKKWEQDPETFLIIIKGAGGKAFCAGGDIRVISEAEKAKQKIAPVFFRE
EYMLNNAVGSCQKPYVALIHGITMGGGVGLSVHGQFRVATEKCLFAMPETAIGLFPDVGG
GYFLPRLQGKLGYFLALTGFRLKGRDVYRAGIATHFVDSEKLAMLEEDLLALKSPSKENI
ASVLENYHTESKIDRDKSFILEEHMDKINSCFSANTVEEIIENLQQDGSSFALEQLKVIN
KMSPTSLKITLRQLMEGSSKTLQEVLTMEYRLSQACMRGHDFHEGVRAVLIDKDQSPKWK
PADLKEVTEEDLNNHFKSLGSSDLKF
|
| External Links |
|---|
| GenBank ID Protein
| 37594471 |
| UniProtKB/Swiss-Prot ID
| Q6NVY1 |
| UniProtKB/Swiss-Prot Entry Name
| HIBCH_HUMAN |
| PDB IDs
|
|
| GenBank Gene ID
| NM_014362.3 |
| GeneCard ID
| HIBCH |
| GenAtlas ID
| HIBCH |
| HGNC ID
| HGNC:4908 |
| References |
|---|
| General References
| Not Available |