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GAA anticorps (AA 541-640) (Alexa Fluor 350)

GAA Reactivité: Humain, Souris, Rat WB, IF (cc), IF (p) Hôte: Lapin Polyclonal Alexa Fluor 350
N° du produit ABIN1693849
  • Antigène Voir toutes GAA Anticorps
    GAA (Glucosidase, Alpha, Acid (GAA))
    Épitope
    • 14
    • 9
    • 6
    • 6
    • 4
    • 3
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 541-640
    Reactivité
    • 47
    • 21
    • 20
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    Humain, Souris, Rat
    Hôte
    • 47
    • 4
    Lapin
    Clonalité
    • 47
    • 4
    Polyclonal
    Conjugué
    • 27
    • 5
    • 4
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp GAA est conjugé à/à la Alexa Fluor 350
    Application
    • 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))
     Réactivité croisée
    Humain, Souris, Rat
    Homologie
    Dog,Sheep,Pig,Horse
    Purification
    Purified by Protein A.
    Immunogène
    KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha
    Isotype
    IgG
    Top Product
    Discover our top product GAA Anticorps primaire
  • Indications d'application
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    Restrictions
    For Research Use only
  • Format
    Liquid
    Concentration
    1 μg/μL
    Buffer
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    Agent conservateur
    ProClin
    Précaution d'utilisation
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Stock
    -20 °C
    Stockage commentaire
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Date de péremption
    12 months
  • Antigène
    GAA (Glucosidase, Alpha, Acid (GAA))
    Autre désignation
    GAA (GAA Produits)
    Synonymes
    anticorps LYAG, anticorps E430018M07Rik, anticorps glucosidase alpha, acid, anticorps glucosidase, alpha, acid, anticorps transmembrane and coiled-coil domain family 1, anticorps glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II), anticorps GAA, anticorps Gaa, anticorps TMCC1, anticorps gaa
    Sujet

    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].

    ID gène
    2548
    UniProt
    P10253
    Pathways
    Cellular Glucan Metabolic Process
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