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Protein

Hydroxyacid-oxoacid transhydrogenase, mitochondrial

Gene

Adhfe1

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-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

    Complete GO annotation...

    Keywords - Molecular functioni

    Oxidoreductase

    Enzyme and pathway databases

    ReactomeiR-RNO-880009. Interconversion of 2-oxoglutarate and 2-hydroxyglutarate.
    SABIO-RKQ4QQW3.

    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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
    Proteomesi
    • UP000002494 Componenti: Chromosome 5

    Organism-specific databases

    RGDi1308863. Adhfe1.

    Subcellular locationi

    • Mitochondrion 1 Publication

    GO - Cellular componenti

    Complete GO annotation...

    Keywords - Cellular componenti

    Mitochondrion

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini? – 467Hydroxyacid-oxoacid transhydrogenase, mitochondrialPRO_0000322999
    Transit peptidei1 – ?MitochondrionSequence analysis

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei445 – 4451N6-acetyllysineBy similarity
    Modified residuei452 – 4521PhosphoserineCombined sources

    Keywords - PTMi

    Acetylation, Phosphoprotein

    Proteomic databases

    PaxDbiQ4QQW3.
    PRIDEiQ4QQW3.

    PTM databases

    iPTMnetiQ4QQW3.
    PhosphoSiteiQ4QQW3.

    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.
    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

    InterProiIPR001670. ADH_Fe.
    [Graphical view]
    PfamiPF00465. Fe-ADH. 1 hit.
    [Graphical view]

    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.

    Cross-referencesi

    Sequence databases

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

    3D structure databases

    ProteinModelPortaliQ4QQW3.
    ModBaseiSearch...
    MobiDBiSearch...

    Protein-protein interaction databases

    STRINGi10116.ENSRNOP00000009462.

    PTM databases

    iPTMnetiQ4QQW3.
    PhosphoSiteiQ4QQW3.

    Proteomic databases

    PaxDbiQ4QQW3.
    PRIDEiQ4QQW3.

    Protocols and materials databases

    Structural Biology KnowledgebaseSearch...

    Genome annotation databases

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

    Organism-specific databases

    CTDi137872.
    RGDi1308863. Adhfe1.

    Phylogenomic databases

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

    Enzyme and pathway databases

    ReactomeiR-RNO-880009. Interconversion of 2-oxoglutarate and 2-hydroxyglutarate.
    SABIO-RKQ4QQW3.

    Miscellaneous databases

    PROiQ4QQW3.

    Gene expression databases

    BgeeiENSRNOG00000007069.
    GenevisibleiQ4QQW3. RN.

    Family and domain databases

    InterProiIPR001670. ADH_Fe.
    [Graphical view]
    PfamiPF00465. Fe-ADH. 1 hit.
    [Graphical view]
    ProtoNetiSearch...

    Entry informationi

    Entry nameiHOT_RAT
    AccessioniPrimary (citable) accession number: Q4QQW3
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: March 18, 2008
    Last sequence update: July 19, 2005
    Last modified: September 7, 2016
    This is version 68 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. SIMILARITY comments
      Index of protein domains and families

    Similar proteinsi

    Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
    100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
    90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
    50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.