MBL

GAA Rabbit pAb

Product Code:
 
MBL-CNA7674T
Product Group:
 
Primary Antibodies
Supplier:
 
MBL
Host Type:
 
Rabbit
Antibody Isotype:
 
IgG
Antibody Clonality:
 
Polyclonal
Regulatory Status:
 
RUO
Target Species:
  • Human
  • Mouse
  • Rat
Applications:
  • Immunocytochemistry (ICC)
  • Immunofluorescence (IF)
  • Immunoprecipitation (IP)
  • Western Blot (WB)
Shipping:
 
ice pack : 2 to 4°C temperature range.
Storage:
 
Store at -20°C
 

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CodeSizePrice
MBL-CNA7674T100 uL£442.00
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This product comes from: United States.
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Further Information

Application Note:
WB,1:500 - 1:1000 | IF/ICC,1:50 - 1:200 | IP,1:50 - 1:200
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.
Conjugate:
Unconjugated
Formulation:
PBS with 0.01% thimerosal, 50% glycerol
Immunogen:
A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2).
Molecular Weight:
105kDa
Peptide Sequence:
VVQQYLDVVGYPFMPPYWGLGFHLCRWGYSSTAITRQVVENMTRAHFPLDVQWNDLDYMDSRRDFTFNKDGFRDFPAMVQELHQGGRRYMMIVDPAISSSG

Documents