GAA Antibody / Glucosidase alpha acid | R32608

GAA Antibody / Glucosidase alpha acid | R32608 | Gentaur US, UK & Europe Disrtribition

Family: Primary antibody

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

Format: Antigen affinity purified

Clone: N/A

Host Animal: Rabbit

Clonality: Polyclonal (rabbit origin)

Species Reactivity: Human

Application: WB, IHC-P

Buffer: Lyophilized from 1X PBS with 2.5% BSA and 0.025% sodium azide

Limitation: This GAA antibody is available for research use only.

Purity: Antigen affinity

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 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody.

Storage: After reconstitution, the GAA 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.