Product Datasheet
GAA Polyclonal Antibody
Catalog Number: 29700
Technical:tech@swbio.com
Information:info@swbio.com
Description
- Swiss-Prot No.:
- Swiss-Prot#:P10253
NCBI Gene ID:2548
- Form of Antibody:
- Avoid freeze / thaw cycles.|Buffer: PBS with 50% glycerol, pH7.4.
- Immunogen:
- A synthetic peptide of human GAA (NP_000143.2).
- reactivity:
- Human,Mouse,Rat
- appl_detail:
- WB 1:500 - 1:2000
IF 1:50 - 1:200
IP 1:50 - 1:200
- other_names:
- GAA; LYAG; glucosidase alpha, acid
- Purification:
- Affinity purification
- Background:
- 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.
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