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GAA Antikörper (Middle Region)

GAA Reaktivität: Human WB, IHC Wirt: Kaninchen Polyclonal unconjugated
Produktnummer ABIN4886596
  • Target Alle GAA Antikörper anzeigen
    GAA (Glucosidase, Alpha, Acid (GAA))
    Bindungsspezifität
    • 15
    • 7
    • 4
    • 4
    • 4
    • 3
    • 3
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 494-527, Middle Region
    Reaktivität
    • 50
    • 25
    • 23
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    Human
    Wirt
    • 54
    • 6
    Kaninchen
    Klonalität
    • 52
    • 8
    Polyklonal
    Konjugat
    • 40
    • 4
    • 3
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser GAA Antikörper ist unkonjugiert
    Applikation
    • 54
    • 29
    • 18
    • 18
    • 14
    • 13
    • 13
    • 7
    • 4
    • 3
    • 2
    • 1
    Western Blotting (WB), Immunohistochemistry (IHC)
    Verwendungszweck
    Anti-GAA Antibody Picoband®
    Sequenz
    TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR
    Kreuzreaktivität (Details)
    No cross-reactivity with other proteins
    Produktmerkmale
    Anti-GAA Antibody Picoband® (ABIN4886596). Tested in IHC, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
    Aufreinigung
    Immunogen affinity purified.
    Immunogen
    A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.
    Isotyp
    IgG
    Top Product
    Discover our top product GAA Primärantikörper
  • Applikationshinweise
    Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human
    Western blot, 0.1-0.5 μg/mL, Human
    1. "Entrez Gene: GAA glucosidase, alpha, acid (Pompe disease, glycogen storage disease type II)". 2. Donald J. Voet, Judith G. Voet, Charlotte W. Pratt (2008). "Additional Pathways in Carbohydrate Metabolism". Principles of Biochemistry, Third edition. Wiley. p. 538. 3. Reuser AJ, Kroos MA, Hermans MM, et al. (1995). "Glycogenosis type II (acid maltase deficiency).". Muscle Nerve. 3: S61-9.
    Kommentare

    Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).

    Beschränkungen
    Nur für Forschungszwecke einsetzbar
  • Format
    Lyophilized
    Rekonstitution
    Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
    Konzentration
    500 μg/mL
    Buffer
    Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg Sodium azide.
    Konservierungsmittel
    Sodium azide
    Vorsichtsmaßnahmen
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    Handhabung
    Avoid repeated freezing and thawing.
    Lagerung
    4 °C,-20 °C
    Informationen zur Lagerung
    Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
    It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
  • Target
    GAA (Glucosidase, Alpha, Acid (GAA))
    Andere Bezeichnung
    GAA (GAA Produkte)
    Hintergrund

    Synonyms: Lysosomal alpha-glucosidase,3.2.1.20,Acid maltase,Aglucosidase alfa,76 kDa lysosomal alpha-glucosidase,70 kDa lysosomal alpha-glucosidase,GAA,

    Tissue Specificity: Most abundant in heart, brain, liver, skeletal muscle and testis but absent in thymus and peripheral blood leukocytes.

    Background: Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.

    Molekulargewicht
    110 kDa, 95kDa, 76kDa, 70 kDa
    Gen-ID
    2548
    UniProt
    P10253
    Pathways
    Cellular Glucan Metabolic Process
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