α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体

Quantity size:

0.2ml

Concentration:

1mg/ml α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

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 processingα葡萄糖苷酶/溶酶体α-葡糖苷酶抗体. 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].

α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体Also known as:

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.

Specificity:

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Rabbit Polyclonal IgG, α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Dog, Pig, Horse, Sheep, .

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Immunogen: KLH conjugated synthetic peptide derived from human GAA/Gluc.

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Predicted Molecular Weight: 83/92/98kDa.

α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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