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Home >> Antibodies >> Glucosidase Alpha Acid Antibody / GAA

Glucosidase Alpha Acid Antibody / GAA [clone 2G7] (RQ6025)

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  Catalog No Formulation Size Price (USD)  
Image RQ6025 0.5mg/ml if reconstituted with 0.2ml sterile DI water 100 ug 429
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IHC staining of FFPE human prostate cancer with Glucosidase Alpha Acid antibody. HIER: boil tissue sections in pH8 EDTA for 20 min and allow to cool before testing.
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IHC staining of FFPE human prostate cancer with Glucosidase Alpha Acid antibody. HIER: boil tissue sections in pH8 EDTA for 20 min and allow to cool before testing.
IHC staining of FFPE human breast cancer with Glucosidase Alpha Acid antibody. HIER: boil tissue sections in pH8 EDTA for 20 min and allow to cool before testing.
IHC staining of FFPE human lung cancer with Glucosidase Alpha Acid antibody. HIER: boil tissue sections in pH8 EDTA for 20 min and allow to cool before testing.
Immunofluorescent staining of FFPE human A549 cells with Glucosidase Alpha Acid antibody antibody (red) and DAPI nuclear stain (blue). HIER: steam section in pH6 citrate buffer for 20 min.
Immunofluorescent staining of FFPE human A549 cells with Glucosidase Alpha Acid antibody antibody (red) and DAPI nuclear stain (blue). HIER: steam section in pH6 citrate buffer for 20 min.
Western blot testing of human 1) A549, 2) HEK293 and 3) PC-3 lysate with Glucosidase Alpha Acid antibody. Expected molecular weight ~110 kDa (precursor), ~95 kDa (intermediate), ~76 and 70 kDa (lysosomal forms).
Flow cytometry testing of human ThP-1 cells with Glucosidase Alpha Acid antibody at 1ug/million cells (blocked with goat sera); Red=cells alone, Green=isotype control, Blue= Glucosidase Alpha Acid antibody.
Availability 1-3 business days
Species Reactivity Human
Format Purified
Clonality Monoclonal (mouse origin)
Isotype Mouse IgG2b
Clone Name 2G7
Purity Affinity purified
Buffer Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
UniProt P10253
Localization Cytoplasmic, membranous
Applications Western blot : 0.5-1ug/ml
Immunohistochemistry : 1-2ug/ml
Immunofluorescence : 2-4ug/ml
Flow cytometry : 1-3ug/million cells
Limitations This Glucosidase Alpha Acid antibody is available for research use only.
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Description

Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.

Application Notes

Optimal dilution of the Glucosidase Alpha Acid antibody should be determined by the researcher.

Immunogen

Amino acids TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR from the human protein were used as the immunogen for the Glucosidase Alpha Acid antibody.

Storage

After reconstitution, the Glucosidase Alpha Acid antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

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