Rabbit Polyclonal Antibody to GAA (N-term)

包装: 1G|100G|500G|1KG|100KG

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别名:Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
应用:WB,IHC
反应种属:Human, Mouse, Rat
规格：50μl/100μl

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.

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.

Specification
Aliases	Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Entrez GeneID	2548
Swissprot	P10253
WB Predicted band size	105.3kDa
Host/Isotype	Rabbit IgG
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity	Human, Mouse, Rat
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.
Formulation	Purified polyclonal antibody supplied in PBS with 0.05% sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Application
WB	1/1000
IHC	1/500

	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.
	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.
	Immunohistochemical analysis of paraffin-embedded Human placenta tissue using P34660 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.
	Immunohistochemical analysis of paraffin-embedded Human liver tissue using P34660 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.