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 |
coenzyme binding |
cofactor binding |
phosphogluconate dehydrogenase (decarboxylating) activity |
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 |
Molecular Function |
3-hydroxyisobutyrate dehydrogenase activity |
NAD binding |
phosphogluconate dehydrogenase (decarboxylating) activity |
Process |
glucose metabolic process |
oxidation reduction |
glucose catabolic 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 |
|
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 |