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GAA Antibody (N-term)

AMM04653G Product Datasheet Protocol

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AMM04653G		100 μl / $495

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Leading Biology Inc.

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GAA Antibody (N-term)

Brand	Leading Biology	Catalog Number	AMM04653G
Product Type	Polyclonal Antibodies	Field of Research
Product Overview	We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format. We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team. This product is a high quality GAA antibody (N-term).
Molecular Weight	105324 Da
Cellular Localization	Antigen Cellular Localization: Lysosome. Lysosome membrane
Host	Rabbit
Species Reactivity	Human
Immunogen	174-203 aa
Target	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.
Isotype	Rabbit Ig
GeneID	2548
UniProt ID	P10253
Function	Essential for the degradation of glygogen to glucose in lysosomes.
Summary	This gene encodes acid alpha-glucosidase, which isessential for the degradation of glycogen to glucose in lysosomes.Different forms of acid alpha-glucosidase are obtained byproteolytic processing. Defects in this gene are the cause ofglycogen storage disease II, also known as Pompe's disease, whichis an autosomal recessive disorder with a broad clinical spectrum.Three transcript variants encoding the same protein have been foundfor this gene.
Form	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.
Storage & Stability	Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.
Applications	WB, IHC, IHC-P, E
Dilution	IHC~~1:25 WB~~1:1000 IHC-P~~1:10~50
Synonyms	Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Images	AMM04653G staining GAA in Human liver tissue sections by Immunohistochemistry (IHC-P - paraformaldehyde-fixed, paraffin-embedded sections). Tissue was fixed with formaldehyde and blocked with 3% BSA for 0. 5 hour at room temperature; antigen retrieval was by heat mediation with a citrate buffer (pH6). Samples were incubated with primary antibody (1/25) for 1 hours at 37°C. A undiluted biotinylated goat polyvalent antibody was used as the secondary antibody. Western blot analysis of lysate from Jurkat cell line, using GAA Antibody (N-term)( AMM04653G). AMM04653G 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. Western blot analysis of lysates from A549, MCF-7, SW620, PC-3 cell line (from left to right), using GAA Antibody (N-term)( AMM04653G). AMM04653G was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:10000 dilution was used as the secondary antibody. Lysates at 20ug per lane.
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