GAA Antikörper

Produktnummer ABIN7880880

Der Maus Monoklonal anti-GAA Antikörper wird verwendet zum Nachweis von GAA in Proben von Human. Er wurde validiert für WB, IHC (p) und IF.

Kurzübersicht für GAA Antikörper (ABIN7880880)

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

Reaktivität: Human

Wirt: Maus

Klonalität: Monoklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunofluorescence (IF)

Klon: 2G7

Produktdetails anti-GAA Antikörper

Verwendungszweck: Glucosidase Alpha Acid Antibody / GAA

Sequenz: TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR

Aufreinigung: Affinity purified

Immunogen: Amino acids TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR from the human protein were used as the immunogen for the Glucosidase Alpha Acid antibody.

Isotyp: IgG2b

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the Glucosidase Alpha Acid antibody should be determined by the researcher.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Buffer: 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: After reconstitution, the Glucosidase Alpha Acid antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Details zu GAA

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

Andere Bezeichnung: Glucosidase Alpha Acid

Hintergrund: 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.

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process