GAA Antibody / Glucosidase alpha acid

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NSJ Bioreagents
Product Code: NSJ-RQ6025
Product Group: Primary Antibodies
Supplier: NSJ Bioreagents
CodeSizePrice
NSJ-RQ6025-100ug100 ug£535.00
Quantity:
Prices exclude any Taxes / VAT

Overview

Host Type: Mouse
Antibody Isotype: Mouse IgG2b
Antibody Clonality: Monoclonal
Antibody Clone: 2G7
Regulatory Status: RUO
Target Species: Human
Applications:
  • Fluorescence-activated cell sorting (FACS)
  • Immunofluorescence (IF)
  • Immunohistochemistry- Paraffin Embedded (IHC-P)
  • Western Blot (WB)
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.

Images

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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 breast cancer with Glucosidase Alpha Acid antibody. HIER: boil tissue sections in pH8 EDTA for 20 min and allow to cool before testing.
3 / 7
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.
4 / 7
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.
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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.
6 / 7
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).
7 / 7
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.
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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.

Documents

Further Information

Application Details :
Western blot: 0.5-1ug/ml,Immunohistochemistry: 1-2ug/ml,Immunofluorescence: 2-4ug/ml,Flow cytometry: 1-3ug/million cells
Application Note:
Optimal dilution of the Glucosidase Alpha Acid antibody should be determined by the researcher.
Buffer:
Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
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.
Format :
Purified
Formulation :
0.5mg/ml if reconstituted with 0.2ml sterile DI water
Immunogen:
Amino acids TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR from the human protein were used as the immunogen for the Glucosidase Alpha Acid antibody.
Limitation:
This Glucosidase Alpha Acid antibody is available for research use only.
Localization:
Cytoplasmic, membranous
Purity:
Affinity purified
Species Reactivity :
Human
Uniprot #:
P10253