GAA Antikörper (Middle Region) (HRP)

Produktnummer ABIN7993505

Der HRP-konjugierte Kaninchen Polyklonal anti-GAA Antikörper (ABIN7993505) detektiert spezifisch GAA in WB, IHC und ELISA. Dieser Antikörper reagiert spezifisch mit Proben aus Human.

Kurzübersicht für GAA Antikörper (Middle Region) (HRP) (ABIN7993505)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GAA Antikörper ist konjugiert mit HRP

Applikation: Western Blotting (WB), Immunohistochemistry (IHC), ELISA

Produktdetails anti-GAA Antikörper (HRP)

Bindungsspezifität: Middle Region

Verwendungszweck: Anti-GAA Antibody HRP Conjugated

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.

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

Anwendungsinformationen

Applikationshinweise: Western blot, Optimal dilutions should be determined by end users. Immunohistochemistry (Paraffin-embedded Section), Optimal dilutions should be determined by end users. ELISA, Optimal dilutions should be determined by end users.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4.

Lagerung: -20 °C

Informationen zur Lagerung: At -20°C for one year from date of receipt. Avoid repeated freezing and thawing.

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

Gen-ID: 2548

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process