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GAA, NT (GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76kD lysosomal alpha-glucosidase, 70kD lysosomal alpha-glucosidase)
品牌： MyBioSource
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对比
咨询反应种属：
Human
实验应用：
ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
产品介绍
产品介绍
产品信息
纯化方式
Affinity Purified
Purified by Protein A affinity chromatography.
免疫原
GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 173-203 amino acids from the N-terminal region of human GAA.
商品描述
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.
形式
Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
产品类型
Polyclonal
应用
实验应用
ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC)
反应种属
Human
背景
别名
Anti -GAA, NT (GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76kD lysosomal alpha-glucosidase, 70kD lysosomal alpha-glucosidase)
制备和贮存
保存方式
May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
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