GAA Antibody / Glucosidase alpha acid | RQ6025

GAA Antibody / Glucosidase alpha acid | RQ6025 | Gentaur US, UK & Europe Disrtribition

Family: Primary antibody

Formulation: 0.5mg/ml if reconstituted with 0.2ml sterile DI water

Format: Purified

Clone: 2G7

Host Animal: Mouse

Clonality: Monoclonal (mouse origin)

Species Reactivity: Human

Application: WB, IHC-P, IF, FACS

Buffer: Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide

Limitation: This Glucosidase Alpha Acid antibody is available for research use only.

Purity: Affinity purified

Description: 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.

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

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