Hydroxyacid-oxoacid transhydrogenase, mitochondrial
Details
- Name
- Hydroxyacid-oxoacid transhydrogenase, mitochondrial
- Synonyms
- 1.1.99.24
- ADHFe1
- Alcohol dehydrogenase iron-containing protein 1
- Fe-containing alcohol dehydrogenase
- HOT
- Gene Name
- ADHFE1
- Organism
- Humans
- Amino acid sequence
>lcl|BSEQ0049427|Hydroxyacid-oxoacid transhydrogenase, mitochondrial MAAAARARVAYLLRQLQRAACQCPTHSHTYSQAPGLSPSGKTTDYAFEMAVSNIRYGAAV TKEVGMDLKNMGAKNVCLMTDKNLSKLPPVQVAMDSLVKNGIPFTVYDNVRVEPTDSSFM EAIEFAQKGAFDAYVAVGGGSTMDTCKAANLYASSPHSDFLDYVSAPIGKGKPVSVPLKP LIAVPTTSGTGSETTGVAIFDYEHLKVKIGITSRAIKPTLGLIDPLHTLHMPARVVANSG FDVLCHALESYTTLPYHLRSPCPSNPITRPAYQGSNPISDIWAIHALRIVAKYLKRAVRN PDDLEARSHMHLASAFAGIGFGNAGVHLCHGMSYPISGLVKMYKAKDYNVDHPLVPHGLS VVLTSPAVFTFTAQMFPERHLEMAEILGADTRTARIQDAGLVLADTLRKFLFDLDVDDGL AAVGYSKADIPALVKGTLPQERVTKLAPCPQSEEDLAALFEASMKLY
- Number of residues
- 467
- Molecular Weight
- 50307.42
- Theoretical pI
- Not Available
- GO Classification
- Functionsalcohol dehydrogenase (NAD) activity / hydroxyacid-oxoacid transhydrogenase activity / metal ion bindingProcesses2-oxoglutarate metabolic process / molecular hydrogen transportComponentscytosol / mitochondrial matrix / mitochondrion
- General Function
- Metal ion binding
- 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.
- Pfam Domain Function
- Fe-ADH (PF00465)
- Transmembrane Regions
- Not Available
- Cellular Location
- Mitochondrion
- Gene sequence
>lcl|BSEQ0049428|Hydroxyacid-oxoacid transhydrogenase, mitochondrial (ADHFE1) ATGGCCGCTGCCGCCCGAGCCCGGGTCGCGTACTTGCTGAGGCAACTGCAACGCGCAGCG TGCCAGTGCCCAACTCATTCTCATACTTACTCCCAAGCCCCTGGACTTTCACCTTCTGGG AAAACAACAGATTATGCCTTTGAGATGGCTGTTTCAAATATTAGATATGGAGCAGCAGTT ACAAAGGAAGTAGGAATGGACCTAAAAAACATGGGTGCTAAAAATGTGTGCTTGATGACA GACAAGAACCTCTCCAAGCTCCCTCCTGTGCAAGTAGCTATGGATTCCCTAGTGAAGAAT GGCATCCCCTTTACGGTTTATGATAATGTGAGAGTGGAACCAACGGATTCAAGCTTCATG GAAGCTATTGAGTTTGCCCAAAAGGGAGCTTTTGATGCCTATGTTGCTGTCGGTGGTGGC TCTACCATGGACACCTGTAAGGCTGCTAATCTGTATGCATCCAGCCCTCATTCTGATTTC CTAGATTATGTCAGTGCCCCCATTGGCAAGGGAAAGCCTGTGTCTGTGCCTCTTAAGCCT CTGATTGCAGTGCCAACTACCTCAGGAACCGGGAGTGAAACTACTGGGGTTGCCATTTTT GACTATGAACACTTGAAAGTAAAAATTGGCATCACTTCGAGAGCCATCAAACCCACACTG GGACTGATTGATCCTCTGCACACCCTCCACATGCCTGCCCGAGTGGTCGCCAACAGTGGC TTTGATGTGCTTTGCCATGCCCTGGAGTCATACACCACCCTGCCCTACCACCTGCGGAGC CCCTGCCCTTCAAATCCCATCACACGGCCTGCGTACCAGGGCAGCAACCCAATCAGTGAC ATTTGGGCTATCCACGCGCTGCGGATCGTGGCTAAGTATCTGAAGAGGGCTGTCAGAAAT CCCGATGATCTTGAAGCAAGGTCTCATATGCACTTGGCAAGTGCTTTTGCTGGCATCGGC TTTGGAAATGCTGGTGTTCATCTGTGCCATGGAATGTCTTACCCAATTTCAGGTTTAGTG AAGATGTATAAAGCAAAGGATTACAATGTGGATCACCCACTGGTGCCCCATGGCCTTTCT GTGGTGCTCACGTCCCCAGCGGTGTTCACTTTCACGGCCCAGATGTTTCCAGAGCGACAC CTGGAGATGGCAGAAATACTGGGAGCCGACACCCGCACTGCCAGGATCCAAGATGCAGGG CTGGTGTTGGCAGACACGCTCCGGAAATTCTTATTCGATCTGGATGTTGATGATGGCCTA GCAGCTGTTGGTTACTCCAAAGCTGATATCCCCGCACTAGTGAAAGGAACGCTGCCCCAG GAAAGGGTCACCAAGCTTGCACCCTGTCCCCAGTCAGAAGAGGATCTGGCTGCTCTGTTT GAAGCTTCAATGAAACTGTATTAA
- Chromosome Location
- Not Available
- Locus
- Not Available
- External Identifiers
Resource Link UniProtKB ID Q8IWW8 UniProtKB Entry Name HOT_HUMAN HGNC ID HGNC:16354 - General References
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