GAA Antikörper (Middle Region) (Fluoro647)

Produktnummer ABIN7993519

Der Maus Monoklonal anti-GAA Antikörper wird verwendet zum Nachweis von GAA in Proben von Human. Er wurde validiert für FACS.

Kurzübersicht für GAA Antikörper (Middle Region) (Fluoro647) (ABIN7993519)

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

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser GAA Antikörper ist konjugiert mit Fluoro647

Applikation: Flow Cytometry (FACS)

Klon: 2G7

Produktdetails anti-GAA Antikörper (Fluoro647)

Bindungsspezifität: Middle Region

Verwendungszweck: Anti-GAA Antibody (monoclonal, 2G7) Fluoro647 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: IgG2b

Anwendungsinformationen

Applikationshinweise: Flow Cytometry, 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, 0.02 % 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: -20 °C

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

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