| Identification |
| HMDB Protein ID
| CDBP00387 |
| Secondary Accession Numbers
| Not Available |
| Name
| 3-hydroxyisobutyrate dehydrogenase, mitochondrial |
| Description
| Not Available |
| Synonyms
|
- HIBADH
|
| Gene Name
| HIBADH |
| Protein Type
| Enzyme |
| Biological Properties |
| General Function
| Involved in 3-hydroxyisobutyrate dehydrogenase activity |
| Specific Function
| Not Available |
| GO Classification
|
| Biological Process |
| branched-chain amino acid catabolic process |
| cellular nitrogen compound metabolic process |
| pentose-phosphate shunt |
| valine metabolic process |
| Cellular Component |
| mitochondrial matrix |
| Function |
| 3-hydroxyisobutyrate dehydrogenase activity |
| oxidoreductase activity, acting on ch-oh group of donors |
| oxidoreductase activity |
| binding |
| nucleotide binding |
| catalytic activity |
| nad or nadh binding |
| oxidoreductase activity, acting on the ch-oh group of donors, nad or nadp as acceptor |
| coenzyme binding |
| cofactor binding |
| phosphogluconate dehydrogenase (decarboxylating) activity |
| Molecular Function |
| 3-hydroxyisobutyrate dehydrogenase activity |
| NAD binding |
| phosphogluconate dehydrogenase (decarboxylating) activity |
| Process |
| cellular amino acid and derivative metabolic process |
| cellular amino acid metabolic process |
| branched chain family amino acid metabolic process |
| valine metabolic process |
| metabolic process |
| pentose-phosphate shunt |
| cellular metabolic process |
| small molecule metabolic process |
| alcohol metabolic process |
| monosaccharide metabolic process |
| hexose metabolic process |
| glucose metabolic process |
| oxidation reduction |
| glucose catabolic process |
|
| Cellular Location
|
- Mitochondrion
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | Valine, Leucine and Isoleucine Degradation |    |  | | Beta-Ketothiolase Deficiency |    | Not Available | | 2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency |    | Not Available | | Propionic Acidemia |    | Not Available | | 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency |    | Not Available |
|
| Gene Properties |
| Chromosome Location
| 7 |
| Locus
| 7p15.2 |
| SNPs
| HIBADH |
| Gene Sequence
|
>1011 bp
ATGGCAGCCTCCTTACGGCTCCTCGGAGCTGCCTCCGGTCTCCGGTACTGGAGCCGGCGG
CTGCGGCCGGCAGCCGGCAGCTTTGCAGCGGTGTGTTCTAGGTCAGTGGCTTCAAAGACT
CCAGTTGGATTCATTGGACTGGGCAACATGGGGAATCCAATGGCAAAAAATCTCATGAAA
CATGGCTATCCACTTATTATTTATGATGTGTTCCCTGATGCCTGCAAAGAGTTTCAAGAT
GCAGGTGAACAGGTAGTATCTTCCCCAGCAGATGTTGCTGAAAAAGCTGACAGAATTATT
ACAATGCTGCCCACCAGTATCAATGCAATAGAAGCTTATTCCGGAGCAAATGGGATTCTA
AAAAAAGTGAAGAAGGGCTCATTATTAATAGATTCCAGCACTATTGATCCTGCAGTTTCA
AAAGAATTGGCCAAAGAAGTTGAGAAAATGGGAGCAGTTTTCATGGATGCCCCTGTTTCT
GGTGGTGTAGGAGCTGCACGATCTGGGAACCTCACGTTTATGGTGGGAGGAGTTGAAGAT
GAATTTGCTGCTGCCCAAGAGTTGCTGGGGTGCATGGGCTCCAACGTGGTGTACTGTGGA
GCTGTTGGGACTGGGCAGGCGGCAAAGATCTGCAACAACATGCTGTTAGCTATTAGTATG
ATTGGAACTGCTGAAGCTATGAATCTTGGAATCAGGTTAGGGCTTGACCCAAAACTACTG
GCTAAAATCCTAAATATGAGCTCAGGACGGTGTTGGTCAAGTGACACTTATAATCCTGTA
CCTGGAGTGATGGATGGCGTTCCCTCGGCTAATAACTATCAGGGTGGATTTGGAACAACA
CTCATGGCTAAGGATCTGGGATTGGCACAAGACTCTGCTACCAGCACAAAGAGCCCAATC
CTTCTTGGCAGTCTGGCCCATCAGATCTACAGGATGATGTGTGCAAAGGGCTACTCAAAG
AAAGACTTCTCATCCGTGTTCCAGTTCCTACGAGAGGAGGAGACCTTCTGA
|
| Protein Properties |
| Number of Residues
| 336 |
| Molecular Weight
| 35328.515 |
| Theoretical pI
| 8.129 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>3-hydroxyisobutyrate dehydrogenase, mitochondrial
MAASLRLLGAASGLRYWSRRLRPAAGSFAAVCSRSVASKTPVGFIGLGNMGNPMAKNLMK
HGYPLIIYDVFPDACKEFQDAGEQVVSSPADVAEKADRIITMLPTSINAIEAYSGANGIL
KKVKKGSLLIDSSTIDPAVSKELAKEVEKMGAVFMDAPVSGGVGAARSGNLTFMVGGVED
EFAAAQELLGCMGSNVVYCGAVGTGQAAKICNNMLLAISMIGTAEAMNLGIRLGLDPKLL
AKILNMSSGRCWSSDTYNPVPGVMDGVPSANNYQGGFGTTLMAKDLGLAQDSATSTKSPI
LLGSLAHQIYRMMCAKGYSKKDFSSVFQFLREEETF
|
| External Links |
| GenBank ID Protein
| 21619591 |
| UniProtKB/Swiss-Prot ID
| P31937 |
| UniProtKB/Swiss-Prot Entry Name
| 3HIDH_HUMAN |
| PDB IDs
|
|
| GenBank Gene ID
| BC032324 |
| GeneCard ID
| HIBADH |
| GenAtlas ID
| HIBADH |
| HGNC ID
| HGNC:4907 |
| References |
| General References
| Not Available |