The GAA Polyclonal Antibody (CAB7674) is specifically designed for research involving GAA, a protein associated with lysosomal storage disorders, specifically Pompe disease. This antibody, generated in rabbits, demonstrates high reactivity with human samples and is validated for use in Western blot applications. It binds to the GAA protein, allowing for accurate detection and analysis in various cell types.GAA, also known as acid alpha-glucosidase, plays a crucial role in breaking down glycogen within lysosomes. Mutations in the GAA gene can lead to the accumulation of glycogen within cells, resulting in the development of Pompe disease.
Research into GAA function is essential for understanding the mechanisms underlying this disorder and developing potential therapeutic interventions.The GAA Polyclonal Antibody is an invaluable tool for investigators studying lysosomal storage disorders, Pompe disease, and related conditions. By enabling precise detection and analysis of the GAA protein, this antibody facilitates advancements in research aimed at improving diagnosis, treatment, and management of these complex diseases.
Product Name:
GAA Rabbit Polyclonal Antibody
SKU:
CAB7674
Size:
20uL, 100uL
Isotype:
IgG
Host Species:
Rabbit
Reactivity:
Human,Mouse,Rat
Immunogen:
A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).
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.
Purification Method:
Affinity purification
Gene ID:
2548
Storage Buffer:
Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.01% thimerosal,50% glycerol,pH7.3.
Western blot analysis of various lysates using GAA Rabbit pAb (CAB7674) at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution.Lysates/proteins: 25μg per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit (AbGn00020).Exposure time: 60s.
