Anti-GAA antibody (bs-13254R)

Anti-GAA antibody

DATASHEET

Host:Rabbit

Target Protein:GAA

IR:Immunogen Range:541-640/952

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:2548

Swiss Prot:P10253

Source:KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha:541-640/952 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

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. [provided by RefSeq, Jul 2008].

Size:100ul

Concentration:1mg/ml

Applications:ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:200-800)

Cross Reactive Species:Human
Mouse
Rat
Dog
Pig
Horse
Sheep
.

For research use only. Not intended for diagnostic or therapeutic use.

VALIDATION IMAGES

Paraformaldehyde-fixed, paraffin embedded (Mouse testis); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (GAA) Polyclonal Antibody, Unconjugated (bs-13254R) at 1:400 overnight at 4°C, followed by a conjugated Goat Anti-Rabbit IgG antibody (bs-0295G-FITC) for 90 minutes, and DAPI for nuclei staining.