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GAA Antikörper (Middle Region)

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch GAA in WB und IHC. Er zeigt eine Reaktivität gegenüber Human.
Produktnummer ABIN4886596

Kurzübersicht für GAA Antikörper (Middle Region) (ABIN4886596)

Target

Alle GAA Antikörper anzeigen
GAA (Glucosidase, Alpha, Acid (GAA))

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)
  • Bindungsspezifität

    • 15
    • 7
    • 4
    • 4
    • 4
    • 3
    • 3
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 494-527, Middle Region

    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
  • 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

    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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