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GAA Antibody (N-term)

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

     
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  • 59 - GAA Antibody (N-term) AP12544a
    Immunohistochemical analysis of paraffin-embedded Human placenta tissue using AP12544a performed on the Leica® BOND RXm. Tissue was fixed with formaldehyde at room temperature, antigen retrieval was by heat mediation with a EDTA buffer (pH9. 0). Samples were incubated with primary antibody(1:500) for 1 hours at room temperature. A undiluted biotinylated CRF Anti-Polyvalent HRP Polymer antibody was used as the secondary antibody.
  • 59 - GAA Antibody (N-term) AP12544a
    Immunohistochemical analysis of paraffin-embedded Human liver tissue using AP12544a performed on the Leica® BOND RXm. Tissue was fixed with formaldehyde at room temperature, antigen retrieval was by heat mediation with a EDTA buffer (pH9. 0). Samples were incubated with primary antibody(1:500) for 1 hours at room temperature. A undiluted biotinylated CRF Anti-Polyvalent HRP Polymer antibody was used as the secondary antibody.
  • 1 - GAA Antibody (N-term) AP12544a
    All lanes : Anti-GAA Antibody (N-term) at 1:1000 dilution Lane 1: Hela whole cell lysate Lane 2: MCF-7 whole cell lysate Lane 3: SW620 whole cell lysate Lane 4: A549 whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 105 kDa Blocking/Dilution buffer: 5% NFDM/TBST.
  • 1 - GAA Antibody (N-term) AP12544a
    Anti-GAA Antibody (N-term) at 1:1000 dilution + Human placenta tissue lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 105 kDa Blocking/Dilution buffer: 5% NFDM/TBST.
  • 1 - GAA Antibody (N-term) AP12544a
    Western blot analysis of lysate from Jurkat cell line, using GAA Antibody (N-term)(Cat. #AP12544a). AP12544a was diluted at 1:2000. A goat anti-rabbit IgG H&L(HRP) at 1:10000 dilution was used as the secondary antibody. Lysate at 20ug.
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Product info
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
IHC-P-Leica, IHC, WB, E
Primary Accession P10253
Other Accession NP_000143.2, NP_001073271.1
Reactivity Human
Host Rabbit
Clonality Polyclonal
Isotype Rabbit Ig
Additional info
Gene ID 2548
Other Names Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Target/Specificity 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.
Dilution IHC-P-Leica~~1:500
WB~~1:2000
Format Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsGAA Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name GAA
Function Essential for the degradation of glycogen in lysosomes (PubMed:1856189, PubMed:7717400, PubMed:14695532, PubMed:18429042). Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans (PubMed:29061980).
Cellular Location Lysosome. Lysosome membrane
Research Areas

BACKGROUND

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.

REFERENCES

Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Labrousse, P., et al. Mol. Genet. Metab. 99(4):379-383(2010)
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
Aoyama, Y., et al. J. Hum. Genet. 54(11):681-686(2009)
Maimaiti, M., et al. J. Hum. Genet. 54(8):493-496(2009)

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