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GAA Antikörper (AA 541-640) (Cy3)

GAA Reaktivität: Human, Maus, Ratte WB, IF (cc), IF (p) Wirt: Kaninchen Polyclonal Cy3
Produktnummer ABIN1702929
  • Target Alle GAA Antikörper anzeigen
    GAA (Glucosidase, Alpha, Acid (GAA))
    Bindungsspezifität
    • 14
    • 9
    • 6
    • 6
    • 4
    • 3
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 541-640
    Reaktivität
    • 47
    • 21
    • 20
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    Human, Maus, Ratte
    Wirt
    • 47
    • 4
    Kaninchen
    Klonalität
    • 47
    • 4
    Polyklonal
    Konjugat
    • 27
    • 5
    • 4
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser GAA Antikörper ist konjugiert mit Cy3
    Applikation
    • 47
    • 19
    • 15
    • 12
    • 12
    • 8
    • 7
    • 5
    • 3
    • 2
    • 2
    • 1
    Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Kreuzreaktivität
    Human, Maus, Ratte
    Homologie
    Dog,Sheep,Pig,Horse
    Aufreinigung
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha
    Isotyp
    IgG
    Top Product
    Discover our top product GAA 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
    GAA (Glucosidase, Alpha, Acid (GAA))
    Andere Bezeichnung
    GAA (GAA Produkte)
    Synonyme
    LYAG antikoerper, E430018M07Rik antikoerper, glucosidase alpha, acid antikoerper, glucosidase, alpha, acid antikoerper, transmembrane and coiled-coil domain family 1 antikoerper, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) antikoerper, GAA antikoerper, Gaa antikoerper, TMCC1 antikoerper, gaa antikoerper
    Hintergrund

    Synonyms: 70 kDa lysosomal alpha-glucosidase, Acid alpha glucosidase, Acid maltase, Aglucosidase alfa, Alpha glucosidase, GAA, Glucosidase alpha acid Pompe disease glycogen storage disease type II, Glucosidase alpha acid, Glucosidase alpha, LYAG, LYAG_HUMAN, Lysosomal alpha glucosidase.

    Background: This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008].

    Gen-ID
    2548
    UniProt
    P10253
    Pathways
    Cellular Glucan Metabolic Process
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