GAA Rabbit mAb (AMR11704N)

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.

We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format. We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team. This product is a high quality GAA Rabbit mAb (APR24466N4) .

GAA; LYAG; glucosidase alpha; acid

2548

P10253

Lysosome, Lysosome membrane

WB 1:500 - 1:2000

Liquid

Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.

Calculated MW: 120,100,75kDa Observed MW: 76KDa/105KDa

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2548

https://www.uniprot.org/uniprot/P10253

Email for sequence