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基因美电话027-88411411
Rabbit Monoclonal Antibody
兔单克隆抗体
GAA Rabbit mAb

货号:R05401

  • 规格价格
  • 100 μL 1800
  • 50 μL 1000
货期:
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  • 应用WB
  • 反应物种Human,Mouse,Rat
  • 理论分子量76kDa
  • 表观分子量105kDa
  • 免疫原A synthetic peptide of human GAA
  • 基因ID2548
  • 蛋白编码P10253
  • 同义词Acid maltase; GAA; Glucosidase alpha acid; LYAG;
  • 宿主物种Rabbit
  • 抗体分型IgG
  • 纯化Affinity purification
  • 储存Store at -20℃. Avoid freeze / thaw cycles.
  • 靶点背景This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.
  • 推荐稀释比WB 1:1000-1:5000
  • 产品形式Liquid
  • Buffer 体系PBS with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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