Identification
BMDB Protein ID BMDBP00595
Secondary Accession Numbers None
Name Hydroxyacid-oxoacid transhydrogenase, mitochondrial
Synonyms Not Available
Gene Name ADHFE1
Protein Type Enzyme
Biological Properties
General Function Energy production and conversion
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). L-3-hydroxybutyrate (L-3-OHB) is also a substrate for HOT when using 2-KG as hydrogen acceptor, resulting in the formation of D-2-HG (By similarity).
Pathways Not Available
Reactions Not Available
GO Classification Not Available
Cellular Location Not Available
Gene Properties
Chromosome Location 14
Locus Not Available
SNPs ADHFE1
Gene Sequence
>1401 bp
ATGGCCGCCGCTCGCTCTCGGGTAGTGCACTTGCTGAGGCTTCTGCAACGCGCAGCATGC
CAGTGCCCGAGTCATTCTCATACTTATTCCCAAGCTCCTGGACTTTCACCTTCTGGGAAA
ACAACAGATTATGCCTTCGAGATGGCTGTTTCAACTATTAGATATGGAGCAGGAGTTACA
AAGGAAGTGGGCATGGACCTACAAAGCATGGGTGCTAAAAATGTTTGTTTGATGACAGAC
AAGAACCTCTCCCAACTCCCTCCTGTACAGACAGTGATGGATTCCCTAGTGAAGAATGGC
ATAAACTTTAAGGTTTATGATCATGTGAGAGTGGAACCAACTGATACAAGCTTCATGGAA
GCCATTGAATTTGCCAAAAAGGGAGCTTTCGATGCCTTCCTGGCCGTGGGTGGCGGTTCC
ACCATAGACACCTGTAAAGCCGCTAATCTGTATTCATCCAGCCCTGACTCTGACTTCCTA
GACTACGTCAATGCCCCCATTGGGAAGGGAAAGCCTGTGACTGTGCCTCTGAAACCTCTG
ATCGCAGTTCCAACTACCTCAGGCACTGGGAGCGAAACTACCGGAGTTGCCATTTTTGAC
TACGAACACTTGAAAGTAAAAACTGGCATTGCTTCGCGAGCCATCAAACCCACACTCGGC
CTGATTGATCCTCTACACACCCTGCACATGCCTGAGCGGGTGGTCGCCAACAGTGGCTTC
GATGTGCTCTGCCACGCCCTGGAGTCCTACACTGCCCTCCCATATCACATGCGGAGCCCC
TGCCCTTCAAGCCCTATCACTCGGCCAGCCTACCAGGGCAGCAACCCCATCAGTGACATC
TGGGCAGTCCACGCACTGCGGATCGTTGCCAAGTATCTGAAGAGGGCTATCAGAAATCCT
GATGATCTTGAAGCAAGGTCTAATATGCACTTGGCAAGTGCTTTTGCTGGCATTGGCTTT
GGAAATGCTGGTGTTCATCTATGCCATGGAATGTCTTACCCAATTTCTGGCTTAGTGAAG
ACTTATAAAGCAAAGGATTACAATGTGGATCATCCCCTGGTGCCTCATGGCCTTTCTGTG
GTGCTCACCTCCCCAGCAGTGTTCACATTTACATCACAGATGTTTCCTGAGCGGCACTTG
GAAGTGGCAGAAATATTGGGAGCTGACACCCGCACTGCCAGGAGGCCAGATGCTGGGCCT
GTCTTGGCAGACACGCTGCGGAAATTCTTATTCGATCTGGATGTGGATGACGGCCTGGCT
GCCATTGGCTACTCCAAGGCCGACATCCCCGAGCTGGTGAAAGGAACGCTGCCCCAGGAA
AGAGTCACCAAGCTTGCACCACGCCCTCAGTCAGAAGAGGATCTATCTGCTCTGTTTGAA
GCATCAATGAAACTGTATTAA
Protein Properties
Number of Residues 466
Molecular Weight 50343.0
Theoretical pI 7.65
Pfam Domain Function Not Available
Signals
  • None
Transmembrane Regions Not Available
Protein Sequence
>Hydroxyacid-oxoacid transhydrogenase, mitochondrial
MAAARSRVVHLLRLLQRAACQCPSHSHTYSQAPGLSPSGKTTDYAFEMAVSTIRYGAGVT
KEVGMDLQSMGAKNVCLMTDKNLSQLPPVQTVMDSLVKNGINFKVYDHVRVEPTDTSFME
AIEFAKKGAFDAFLAVGGGSTIDTCKAANLYSSSPDSDFLDYVNAPIGKGKPVTVPLKPL
IAVPTTSGTGSETTGVAIFDYEHLKVKTGIASRAIKPTLGLIDPLHTLHMPERVVANSGF
DVLCHALESYTALPYHMRSPCPSSPITRPAYQGSNPISDIWAVHALRIVAKYLKRAIRNP
DDLEARSNMHLASAFAGIGFGNAGVHLCHGMSYPISGLVKTYKAKDYNVDHPLVPHGLSV
VLTSPAVFTFTSQMFPERHLEVAEILGADTRTARRPDAGPVLADTLRKFLFDLDVDDGLA
AIGYSKADIPELVKGTLPQERVTKLAPRPQSEEDLSALFEASMKLY
GenBank ID Protein AAI49098.1
UniProtKB/Swiss-Prot ID A6QP15
UniProtKB/Swiss-Prot Entry Name HOT_BOVIN
PDB IDs Not Available
GenBank Gene ID BC149097
GeneCard ID ADHFE1
GenAtlas ID Not Available
HGNC ID Not Available
References
General References Not Available