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GAA Antibody

Catalog Number:OM279346
OM279346
Discount Price
OM279346-100ul
1~2 Week
100ul
OM279346-50ul
50ul
OM279346-20ul
20ul
1~2 Week
Product Profile
Product Name GAA Antibody
Antibody Type Primary Antibodies
Antigen Alias Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Product description This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.1) Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Immunogen This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.
Key Feature
Clonality Polyclonal
Isotype Ig
Host Species Rabbit
Tested Applications IHCIHC-PWB
For IHC starting dilution is: 1:25 For WB starting dilution is: 1:1000:
Species Reactivity Human
Concentration 1mg/ml
Purification Affinity purified
Target Information
Gene Symbol GAA
Alternative Names Lysosomal alpha-glucosidase
Acid maltase
Aglucosidase alfa
76 kDa lysosomal alpha-glucosidase
70 kDa lysosomal alpha-glucosidase
GAA
Molecular Weight(MW) 105 kDa
Database Links
Entrez Gene 2548
Protein Accession P10253
Application

Application

Western blot analysis in HL-60 cell line lysates (35ug/lane).

Application

GAA Antibody immunohistochemistry analysis in formalin fixed and paraffin embedded human lung carcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining.

Application

Western blot analysis of lysates from A549, MCF-7, SW620, PC-3 cell line (from left to right), using GAA Antibody at 1:1000 at each lane.

Application

Western blot analysis of lysate from Jurkat cell line, using GAA Antibody at 1:2000.

Application

Antibody staining GAA in Human liver tissue sections by Immunohistochemistry (IHC-P - paraformaldehyde-fixed, paraffin-embedded sections).
Application Notes For IHC starting dilution is: 1:25 For WB starting dilution is: 1:1000:
Additional Information
Form Liquid
Storage Instructions Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Storage Buffer Supplied in PBS with 0.09% (W/V) sodium azide.
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