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Rabbit Polyclonal Antibody to GAA (N-term)
货号: P34660
别名: Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
应用: WB,IHC
反应种属: Human, Mouse, Rat
抗体类型: Primary antibody
Swissprot: P10253
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Description

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.

Specification

Aliases Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Entrez GeneID 2548
Swissprot P10253
WB Predicted band size 105.3kDa
Host/Isotype Rabbit IgG
Antibody Type Primary antibody
Storage Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity Human, Mouse, Rat
Immunogen This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.
Formulation Purified antibody in PBS with 0.05% sodium azide.

Application

WB 1/1000
IHC 1/500

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