| Identification |
|---|
| HMDB Protein ID
| CDBP02373 |
| Secondary Accession Numbers
| Not Available |
| Name
| Hydroxyacid-oxoacid transhydrogenase, mitochondrial |
| Description
| Not Available |
| Synonyms
|
- ADHFe1
- Alcohol dehydrogenase iron-containing protein 1
- Fe-containing alcohol dehydrogenase
- HOT
|
| Gene Name
| ADHFE1 |
| Protein Type
| Enzyme |
| Biological Properties |
|---|
| General Function
| Involved in oxidoreductase activity |
| Specific Function
| Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate (L-3-OHB) are also substrates for HOT with 10-fold lower activities.
|
| GO Classification
|
| Biological Process |
| 2-oxoglutarate metabolic process |
| molecular hydrogen transport |
| Cellular Component |
| mitochondrial matrix |
| Function |
| oxidoreductase activity |
| ion binding |
| cation binding |
| metal ion binding |
| binding |
| catalytic activity |
| Molecular Function |
| metal ion binding |
| hydroxyacid-oxoacid transhydrogenase activity |
| Process |
| metabolic process |
| oxidation reduction |
|
| Cellular Location
|
- Mitochondrion
|
| Pathways
|
Not Available
|
| Gene Properties |
|---|
| Chromosome Location
| 8 |
| Locus
| 8q13.1 |
| SNPs
| ADHFE1 |
| Gene Sequence
|
>1404 bp
ATGGCCGCTGCCGCCCGAGCCCGGGTCGCGTACTTGCTGAGGCAACTGCAACGCGCAGCG
TGCCAGTGCCCAACTCATTCTCATACTTACTCCCAAGCCCCTGGACTTTCACCTTCTGGG
AAAACAACAGATTATGCCTTTGAGATGGCTGTTTCAAATATTAGATATGGAGCAGCAGTT
ACAAAGGAAGTAGGAATGGACCTAAAAAACATGGGTGCTAAAAATGTGTGCTTGATGACA
GACAAGAACCTCTCCAAGCTCCCTCCTGTGCAAGTAGCTATGGATTCCCTAGTGAAGAAT
GGCATCCCCTTTACGGTTTATGATAATGTGAGAGTGGAACCAACGGATTCAAGCTTCATG
GAAGCTATTGAGTTTGCCCAAAAGGGAGCTTTTGATGCCTATGTTGCTGTCGGTGGTGGC
TCTACCATGGACACCTGTAAGGCTGCTAATCTGTATGCATCCAGCCCTCATTCTGATTTC
CTAGATTATGTCAGTGCCCCCATTGGCAAGGGAAAGCCTGTGTCTGTGCCTCTTAAGCCT
CTGATTGCAGTGCCAACTACCTCAGGAACCGGGAGTGAAACTACTGGGGTTGCCATTTTT
GACTATGAACACTTGAAAGTAAAAATTGGTATCACTTCGAGAGCCATCAAACCCACACTG
GGACTGATTGATCCTCTGCACACCCTCCACATGCCTGCCCGAGTGGTCGCCAACAGTGGC
TTTGATGTGCTTTGCCATGCCCTGGAGTCATACACCACCCTGCCCTACCACCTGCGGAGC
CCCTGCCCTTCAAATCCCATCACACGGCCTGCGTACCAGGGCAGCAACCCAATCAGTGAC
ATTTGGGCTATCCACGCGCTGCGGATCGTGGCTAAGTATCTGAAGAGGGCCGTCAGAAAT
CCCGATGATCTTGAAGCAAGGTCTCATATGCACTTGGCAAGTGCTTTTGCTGGCATCGGC
TTTGGAAATGCTGGTGTTCATCTGTGCCATGGAATGTCTTACCCAATTTCAGGTTTAGTG
AAGATGTATAAAGCAAAGGATTACAATGTGGATCACCCACTGGTGCCCCATGGCCTTTCT
GTGGTGCTCACGTCCCCAGCGGTGTTCACTTTCACGGCCCAGATGTTTCCAGAGCGACAC
CTGGAGATGGCAGAAATATTGGGAGCCGACACCCGCACTGCCAGGATCCAAGATGCAGGG
CTGGTGTTGGCAGACACGCTCCGGAAATTCTTATTCGATCTGGATGTTGATGATGGCCTA
GCAGCTGTTGGTTACTCCAAAGCTGATATCCCCGCACTAGTGAAAGGAACGCTGCCCCAG
GAAAGGGTCACCAAGCTTGCACCCTGTCCCCAGTCAGAAGAGGATCTGGCTGCTCTGTTT
GAAGCTTCAATGAAACTGTATTAA
|
| Protein Properties |
|---|
| Number of Residues
| 467 |
| Molecular Weight
| 50307.42 |
| Theoretical pI
| 7.762 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Hydroxyacid-oxoacid transhydrogenase, mitochondrial
MAAAARARVAYLLRQLQRAACQCPTHSHTYSQAPGLSPSGKTTDYAFEMAVSNIRYGAAV
TKEVGMDLKNMGAKNVCLMTDKNLSKLPPVQVAMDSLVKNGIPFTVYDNVRVEPTDSSFM
EAIEFAQKGAFDAYVAVGGGSTMDTCKAANLYASSPHSDFLDYVSAPIGKGKPVSVPLKP
LIAVPTTSGTGSETTGVAIFDYEHLKVKIGITSRAIKPTLGLIDPLHTLHMPARVVANSG
FDVLCHALESYTTLPYHLRSPCPSNPITRPAYQGSNPISDIWAIHALRIVAKYLKRAVRN
PDDLEARSHMHLASAFAGIGFGNAGVHLCHGMSYPISGLVKMYKAKDYNVDHPLVPHGLS
VVLTSPAVFTFTAQMFPERHLEMAEILGADTRTARIQDAGLVLADTLRKFLFDLDVDDGL
AAVGYSKADIPALVKGTLPQERVTKLAPCPQSEEDLAALFEASMKLY
|
| External Links |
|---|
| GenBank ID Protein
| Not Available |
| UniProtKB/Swiss-Prot ID
| Q8IWW8 |
| UniProtKB/Swiss-Prot Entry Name
| HOT_HUMAN |
| PDB IDs
|
Not Available |
| GenBank Gene ID
| AY033237 |
| GeneCard ID
| ADHFE1 |
| GenAtlas ID
| ADHFE1 |
| HGNC ID
| HGNC:16354 |
| References |
|---|
| General References
| Not Available |