Product Datasheet  
GAA Rabbit mAb  
Catalog Number: 77643  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • P10253
  • Form of Antibody：  
  • Storage：  
  • Store at -20℃. Avoid freeze / thaw cycles.|Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
  • Immunogen：  
  • Recombinant protein of human GAA.
  • reactivity：  
  • Human,Mouse,Rat
  • appl_detail：  
  • WB 1:500 - 1:2000
  • other_names：  
  • GAA; LYAG; glucosidase alpha, acid
  • Purification：  
  • Affinity purification
  • Specificity：  
  • Applications：  
  • WB
  • 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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