bsm-54735R [Primary Antibody]
GAA Monoclonal Antibody
www.biossusa.com
[email protected]
800.501.7654 [DOMESTIC]
+1.781.569.5821 [INTERNATIONAL]
DATASHEET

Host: Rabbit

Target Protein: GAA

Clonality: Monoclonal

Isotype: IgG

Entrez Gene: 2548

Swiss Prot: P10253

Source: Synthetic peptide within Human GAA.

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C for 12 months.

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: 1ug/ul

Predicted Molecular Weight: Predicted band size: 105/76/70 .


Cross Reactive Species: Human

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

VALIDATION IMAGES

Paraformaldehyde-fixed, paraffin embedded Human liver; 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 Monoclonal Antibody, Unconjugated (bsm-54735R) at 1:200 for 30 minutes at room temperature, DAB staining.


Paraformaldehyde-fixed, paraffin embedded Human liver cancer; 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 Monoclonal Antibody, Unconjugated (bsm-54735R) at 1:50 for 30 minutes at room temperature, DAB staining.


Paraformaldehyde-fixed, paraffin embedded Human placenta; 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 Monoclonal Antibody, Unconjugated (bsm-54735R) at 1:50 for 30 minutes at room temperature, DAB staining.


Immunofluorescence staining of paraffin- embedded human liver tissue using anti-Rubisco activase rabbit polyclonal antibody.The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes.(sodium citrate buffer (pH6) for 20 mins.) The tissues were blocked in 10% negative goat serum for 1 hour at room temperature, washed with PBS, and then probed with bsm-54735R at 1/50 dilution for 10 hours at 4℃ and detected using Alexa Fluor® 488 conjugate-Goat anti-Rabbit IgG (H+L) Secondary Antibody at a dilution of 1:500 for 1 hour at room temperature.


HepG2 cells were fixed,permeabilized and incubated in 5% BSA blocking buffer for 30 min at room temperature. Cells were then stained with GAA Monoclonal Antibody(bsm-54735R)at 1:50 dilution in blocking buffer and incubated for 30 min at room temperature, washed twice with 2%BSA in PBS, followed by secondary antibody incubation for 40 min at room temperature. Acquisitions of 20,000 events were performed. Cells stained with primary antibody (red).