Overview |
bs-13254R |
Alpha Glucosidase Polyclonal Antibody |
WB, ELISA, IHC-P, IHC-F, IF(IHC-P), IF(IHC-F), IF(ICC), ICC |
Human, Mouse, Rat |
Dog, Sheep, Pig, Horse |
Specifications |
Unconjugated |
Rabbit |
KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha |
541-640/952 |
Polyclonal |
IgG |
1ug/ul |
Purified by Protein A. |
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
Target |
2548 |
P10253 |
Cytoplasm, Cell membrane |
70 kDa lysosomal alpha-glucosidase; Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; Alpha glucosidase; GAA; Glucosidase alpha acid Pompe disease glycogen storage disease type II; Glucosidase alpha acid; Glucosidase alpha; LYAG; LYAG_HUMAN; Lysosomal alpha glucosidase. |
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]. |
Application Dilution |
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 |