获取电话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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