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Protein

Hydroxyacid-oxoacid transhydrogenase, mitochondrial

Gene

Adhfe1

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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).By similarity

Catalytic activityi

(S)-3-hydroxybutanoate + 2-oxoglutarate = acetoacetate + (R)-2-hydroxyglutarate.
4-hydroxybutanoate + 2-oxoglutarate = succinic semialdehyde + (R)-2-hydroxyglutarate.

Kineticsi

  1. KM=0.3 mM for GHB1 Publication
  2. KM=0.4 mM for D-2-HG1 Publication
  3. KM=0.018 mM for 2-KG1 Publication
  4. KM=0.005 mM for SSA1 Publication
  5. KM=3 mM for L-3-OHB1 Publication

    GO - Molecular functioni

    GO - Biological processi

    Keywordsi

    Molecular functionOxidoreductase

    Enzyme and pathway databases

    ReactomeiR-RNO-880009 Interconversion of 2-oxoglutarate and 2-hydroxyglutarate
    SABIO-RKiQ4QQW3

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Hydroxyacid-oxoacid transhydrogenase, mitochondrial (EC:1.1.99.24)
    Short name:
    HOT
    Alternative name(s):
    Alcohol dehydrogenase iron-containing protein 1
    Short name:
    ADHFe1
    Gene namesi
    Name:Adhfe1
    OrganismiRattus norvegicus (Rat)
    Taxonomic identifieri10116 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeRattus
    Proteomesi
    • UP000002494 Componenti: Chromosome 5

    Organism-specific databases

    RGDi1308863 Adhfe1

    Subcellular locationi

    Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

    Keywords - Cellular componenti

    Mitochondrion

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    ChainiPRO_0000322999? – 467Hydroxyacid-oxoacid transhydrogenase, mitochondrial
    Transit peptidei1 – ?MitochondrionSequence analysis

    Amino acid modifications

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Modified residuei445N6-acetyllysineBy similarity1
    Modified residuei452PhosphoserineCombined sources1

    Keywords - PTMi

    Acetylation, Phosphoprotein

    Proteomic databases

    PaxDbiQ4QQW3
    PRIDEiQ4QQW3

    PTM databases

    iPTMnetiQ4QQW3
    PhosphoSitePlusiQ4QQW3

    Expressioni

    Tissue specificityi

    Expressed in kidney and liver.1 Publication

    Gene expression databases

    BgeeiENSRNOG00000007069
    GenevisibleiQ4QQW3 RN

    Interactioni

    Protein-protein interaction databases

    STRINGi10116.ENSRNOP00000009462

    Structurei

    3D structure databases

    ProteinModelPortaliQ4QQW3
    SMRiQ4QQW3
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiKOG3857 Eukaryota
    COG1454 LUCA
    GeneTreeiENSGT00390000003849
    HOGENOMiHOG000243335
    HOVERGENiHBG057032
    InParanoidiQ4QQW3
    KOiK11173
    OMAiRAACQCP
    OrthoDBiEOG091G064B
    PhylomeDBiQ4QQW3
    TreeFamiTF105710

    Family and domain databases

    InterProiView protein in InterPro
    IPR001670 ADH_Fe/gldA
    PfamiView protein in Pfam
    PF00465 Fe-ADH, 1 hit

    Sequencei

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    Q4QQW3-1 [UniParc]FASTAAdd to basket

    « Hide

            10         20         30         40         50
    MAAAARARVT HLLRHLQSTA CQCPTHSHTY SQVPGLSPSG KTTDYAFEMA
    60 70 80 90 100
    VSNIRYGAGV TKEVGMDLQN MGAKNVCLMT DKNLSQLPPV QIVMDSLSKN
    110 120 130 140 150
    GISFQVYDNV RVEPTDGSFM DAIEFAKKGA FDAYVAVGGG STMDTCKAAN
    160 170 180 190 200
    LYACSPHSEF LDYVNAPIGK GKPVTVPLKP LIAVPTTSGT GSETTGVAIF
    210 220 230 240 250
    DYEHLKVKTG IASRAIKPTL GLVDPLHTLH MPCQVVANSG FDVLCHALES
    260 270 280 290 300
    YTAIPYSMRS PCPSNPIQRP AYQGSNPISD IWAVHALRIV AKYLKRAVRN
    310 320 330 340 350
    PDDLEARSSM HLASAFAGIG FGNAGVHLCH GMSYPISGLV KTYKAKEYNV
    360 370 380 390 400
    DHPLVPHGLS VVLTSPAVFT FTAQMFPERH LETAEILGAN IRTAKIQDAG
    410 420 430 440 450
    PVLADALRKF LFDLNVDDGL AALGYSKDDI PSLVKGTLPQ ERVTKLAPRA
    460
    QSEEDLSALF EASMKLY
    Length:467
    Mass (Da):50,226
    Last modified:July 19, 2005 - v1
    Checksum:i97F4EB2243EBE7DB
    GO

    Sequence databases

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    BC097945 mRNA Translation: AAH97945.1
    RefSeqiNP_001020594.1, NM_001025423.1
    UniGeneiRn.17173

    Genome annotation databases

    EnsembliENSRNOT00000009462; ENSRNOP00000009462; ENSRNOG00000007069
    GeneIDi362474
    KEGGirno:362474
    UCSCiRGD:1308863 rat

    Similar proteinsi

    Entry informationi

    Entry nameiHOT_RAT
    AccessioniPrimary (citable) accession number: Q4QQW3
    Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 18, 2008
    Last sequence update: July 19, 2005
    Last modified: March 28, 2018
    This is version 74 of the entry and version 1 of the sequence. See complete history.
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Reference proteome
    UniProt is an ELIXIR core data resource
    Main funding by: National Institutes of Health