GAA Antikörper (Middle Region)

Produktnummer ABIN7993520

Der Maus Monoklonal anti-GAA Antikörper (Klon 2G7) (ABIN7993520) detektiert spezifisch GAA in WB, IHC, ICC und IF. Dieser Antikörper reagiert spezifisch mit Proben aus Human.

Kurzübersicht für GAA Antikörper (Middle Region) (ABIN7993520)

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF)

Güteklasse: Carrier-free

Klon: 2G7

Produktdetails anti-GAA Antikörper

Bindungsspezifität: Middle Region

Verwendungszweck: Anti-GAA Antibody (monoclonal, 2G7)

Spezifität: No cross reactivity with other proteins.

Kreuzreaktivität (Details): No cross-reactivity with other proteins.

Homologie: different from the related mouse sequence by eight amino acids,and from the related rat sequence by six amino acids.

Produktmerkmale: Anti-GAA Antibody.(monoclonal, 2G7). Tested in IF, IHC, ICC, WB applications. This antibody reacts with Human.

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: IgG2b

Anwendungsinformationen

Applikationshinweise: Western blot, 0.1-0.5 μg/mL, Human Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human Immunocytochemistry/Immunofluorescence, 2 μg/mL, Human

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

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, 0.01 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.

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.

Haltbarkeit: 12 months

Details zu GAA

Target: GAA (Glucosidase, Alpha, Acid (GAA))

Andere Bezeichnung: GAA

Hintergrund:

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.

Gene Full Name: alpha glucosidase

Molekulargewicht: 110 kDa, 95 kDa, 76 kDa

Gen-ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process