bs-13254R [Primary Antibody]
Alpha Glucosidase Polyclonal Antibody
www.biossusa.com
[email protected]
800.501.7654 [DOMESTIC]
+1.781.569.5821 [INTERNATIONAL]
DATASHEET

Host: Rabbit

Target Protein: GAA

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

Purification: Purified by Protein A.

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

Storage: Shipped at 4°C. 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: 1ug/ul

Applications: WB(1:300-5000)
ELISA(1:500-1000)
IHC-P(1:200-400)
IHC-F(1:100-500)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)
ICC(1:100-500)

Predicted Molecular Weight: 83/92/98


Cross Reactive Species: Human
Mouse
Rat

Predicted Cross Reactive Species: Dog
Sheep
Pig
Horse

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

PRODUCT SPECIFIC PUBLICATIONS
  • Rauniyar et al. Quantitative Proteomics of Human Fibroblasts with I1061T Mutation in Niemann-Pick C1 (NPC1) Protein Provides Insights into the Disease Pathogenesis. (2015) Mol.Cell.Proteomics. 14:1734-49Read more>>
VALIDATION IMAGES

Paraformaldehyde-fixed, paraffin embedded rat lung; Antigen retrieval by boiling in sodium citrate buffer (pH6) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 30 minutes; Blocking buffer (normal goat serum) at 37°C for 20min; Antibody incubation with Alpha Glucosidase Polyclonal Antibody (bs-13254R) at 1:200 overnight at 4°C, followed by a conjugated secondary.