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ETHE1 Antikörper (AA 191-254) (AbBy Fluor® 594)

ETHE1 Reaktivität: Human IF (cc), IF (p) Wirt: Kaninchen Polyclonal AbBy Fluor® 594
Produktnummer ABIN2812956
  • Target Alle ETHE1 Antikörper anzeigen
    ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))
    Bindungsspezifität
    • 14
    • 6
    • 3
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    AA 191-254
    Reaktivität
    • 41
    • 18
    • 3
    • 2
    • 2
    • 2
    • 2
    Human
    Wirt
    • 39
    • 2
    Kaninchen
    Klonalität
    • 41
    Polyklonal
    Konjugat
    • 17
    • 3
    • 3
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser ETHE1 Antikörper ist konjugiert mit AbBy Fluor® 594
    Applikation
    • 16
    • 12
    • 12
    • 10
    • 8
    • 7
    • 4
    • 3
    • 2
    • 2
    Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Homologie
    Human,Mouse,Dog,Cow,Sheep,Pig,Rabbit
    Aufreinigung
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human E1
    Isotyp
    IgG
    Top Product
    Discover our top product ETHE1 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
    ETHE1 (Ethylmalonic Encephalopathy 1 (ETHE1))
    Andere Bezeichnung
    ETHE1 (ETHE1 Produkte)
    Synonyme
    zgc:85680 antikoerper, HSCO antikoerper, YF13H12 antikoerper, 0610025L15Rik antikoerper, Hsco antikoerper, ETHE1, persulfide dioxygenase antikoerper, ethylmalonic encephalopathy 1 antikoerper, Ethe1 antikoerper, ethe1 antikoerper, ETHE1 antikoerper
    Hintergrund

    Synonyms: mitochondrial, Ethe1, ETHE1 protein, mitochondrial precursor, ETHE1_HUMAN, ethylmalonic encephalopathy 1, Ethylmalonic encephalopathy protein 1, hepatoma subtracted clone one, Hepatoma subtracted clone one protein, HSCO, Protein ETHE1, YF13H12.

    Background: Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53-induced apoptosis by preventing nuclear localization of RELA.Involvement in disease:Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) . EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria.

    Gen-ID
    23474
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