GAA Antikörper (AA 174-203)

Produktnummer ABIN7871285

Dieser Kaninchen Polyklonal Antikörper detektiert spezifisch GAA in WB und IHC (p). Es zeigt Reaktivität gegenüber Proben von Human.

Kurzübersicht für GAA Antikörper (AA 174-203) (ABIN7871285)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GAA Antikörper ist unkonjugiert

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

Produktdetails anti-GAA Antikörper

Bindungsspezifität: AA 174-203

Verwendungszweck: GAA Antibody / Glucosidase alpha acid

Aufreinigung: Antigen affinity purified

Immunogen: A portion of amino acids 174-203 from the human protein were used as the immunogen for the GAA antibody.

Isotyp: Ig Fraction

Anwendungsinformationen

Applikationshinweise: The stated application concentrations are suggested starting points. Titration of the GAA antibody may be required due to differences in protocols and secondary/substrate sensitivity.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: In 1X PBS, pH 7.4, with 0.09 % 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: Aliquot the GAA antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.

Details zu GAA

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

Andere Bezeichnung: GAA

Hintergrund: This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.

UniProt: P10253

Pathways: Cellular Glucan Metabolic Process