Description
GAA Antibody / Glucosidase alpha acid | F54279-0.4ML | Gentaur US, UK & Europe Disrtribition
Family: Primary antibody
Formulation: In 1X PBS, pH 7.4, with 0.09% sodium azide
Format: Purified
Clone: N/A
Host Animal: Rabbit
Clonality: Polyclonal (rabbit origin)
Species Reactivity: Human
Application: WB, IHC-P
Buffer: N/A
Limitation: This GAA antibody is available for research use only.
Purity: Antigen affinity purified
Description: 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.
Immunogen: A portion of amino acids 174-203 from the human protein were used as the immunogen for the GAA antibody.
Storage: Aliquot the GAA antibody and store frozen at -20 °C or colder. Avoid repeated freeze-thaw cycles.