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HIBADH Antikörper (AA 251-336) (AbBy Fluor® 350)

HIBADH Reaktivität: Human, Maus WB, IF (cc), IF (p) Wirt: Kaninchen Polyclonal AbBy Fluor® 350
Produktnummer ABIN1692767
  • Target Alle HIBADH Antikörper anzeigen
    HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
    Bindungsspezifität
    • 14
    • 14
    • 7
    • 4
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 251-336
    Reaktivität
    • 51
    • 33
    • 11
    • 5
    • 4
    • 4
    • 4
    • 3
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    Human, Maus
    Wirt
    • 49
    • 2
    Kaninchen
    Klonalität
    • 51
    Polyklonal
    Konjugat
    • 22
    • 3
    • 3
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser HIBADH Antikörper ist konjugiert mit AbBy Fluor® 350
    Applikation
    • 41
    • 20
    • 12
    • 12
    • 7
    • 7
    • 3
    • 3
    • 2
    Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Kreuzreaktivität
    Human, Maus
    Homologie
    Rat,Cow,Sheep,Horse
    Aufreinigung
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human HIBADH
    Isotyp
    IgG
    Top Product
    Discover our top product HIBADH Primärantikörper
  • Applikationshinweise
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    Beschränkungen
    Nur für Forschungszwecke einsetzbar
  • Format
    Liquid
    Konzentration
    1 μg/μL
    Buffer
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    Konservierungsmittel
    ProClin
    Vorsichtsmaßnahmen
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Lagerung
    -20 °C
    Informationen zur Lagerung
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Haltbarkeit
    12 months
  • Target
    HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))
    Andere Bezeichnung
    Hibadh (HIBADH Produkte)
    Synonyme
    NS5ATP1 antikoerper, 6430402H10Rik antikoerper, AI265272 antikoerper, hibadh antikoerper, zgc:66262 antikoerper, wu:fb07f02 antikoerper, zgc:100804 antikoerper, 3-hydroxyisobutyrate dehydrogenase antikoerper, 3-hydroxyisobutyrate dehydrogenase b antikoerper, 3-hydroxyisobutyrate dehydrogenase S homeolog antikoerper, 3-hydroxyisobutyrate dehydrogenase a antikoerper, HIBADH antikoerper, Hibadh antikoerper, hibadhb antikoerper, hibadh.S antikoerper, hibadha antikoerper
    Hintergrund

    Synonyms: 3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase, 3 hydroxyisobutyrate dehydrogenase, 3 hydroxyisobutyrate dehydrogenase mitochondrial, EC 1.1.1.31, MGC40361, NS5ATP1, 3HIDH_HUMAN.

    Background: HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7.0 and 10.0, with optimal activity between 8.8 and 9.0. It was previously hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.

    Gen-ID
    11112
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