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ATXN3L Antikörper (AA 251-355) (AbBy Fluor® 750)

ATXN3L Reaktivität: Human WB, IF (cc), IF (p) Wirt: Kaninchen Polyclonal AbBy Fluor® 750
Produktnummer ABIN4997069
  • Target Alle ATXN3L Antikörper anzeigen
    ATXN3L (Ataxin 3-Like (ATXN3L))
    Bindungsspezifität
    AA 251-355
    Reaktivität
    • 17
    • 1
    Human
    Wirt
    • 17
    Kaninchen
    Klonalität
    • 17
    Polyklonal
    Konjugat
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser ATXN3L Antikörper ist konjugiert mit AbBy Fluor® 750
    Applikation
    • 17
    • 12
    • 12
    • 3
    • 3
    • 2
    • 1
    Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Kreuzreaktivität
    Human
    Aufreinigung
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human ATXN3L
    Isotyp
    IgG
    Top Product
    Discover our top product ATXN3L 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
    ATXN3L (Ataxin 3-Like (ATXN3L))
    Andere Bezeichnung
    ATXN3L (ATXN3L Produkte)
    Synonyme
    MJDL antikoerper, ataxin 3 like antikoerper, ATXN3L antikoerper
    Hintergrund

    Synonyms: ATX3L_HUMAN, ATXN3L, Machado-Joseph disease protein 1-like, MJDL, Putative ataxin-3-like protein.

    Background: Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) , also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.

    Gen-ID
    92552
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