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Anti-GAA Antibody (Monoclonal, 2G7)
Anti-GAA Antibody (Monoclonal, 2G7)
- 中文名称：
- Anti-GAA Antibody (Monoclonal, 2G7)
- 英文名称：
- Anti-GAA Antibody (Monoclonal, 2G7)
- 品牌：
- AAA Biotech
- 品牌介绍：
- AAA Biotech专注于为全球生命科学研究提供高品质的蛋白质研究工具，核心产品包括经严格验证的抗体、重组蛋白及ELISA试剂盒。
- 货号：
- AAA19211
- 规格：
- 0.01 mg|0.1 mg|0.1 mg (Carrier Free)|0.1 mg (HRP)|0.1 mg (FITC)
- 保存建议：
- Store at -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen store at -20 degree C for 6 months. Avoid repeated freezing and thawing.
- 货期：
- 6-8周
- 来源宿主：
- Mouse
- 反应种属：
- Human
- 应用：
- WB (Western Blot), IHC (Immunohistochemistry), ICC (Immunocytochemistry), IF (Immunofluorescence), FCM/FACS (Flow Cytometry)
- 免责声明：
- *本产品仅供科研实验使用，不得用于临床诊断。*
- 其他：
克隆性:Monoclonal
同型:IgG2b
克隆号:[2G7]
特异性:N/A
纯度:Immunogen affinity purified.
形式：Lyophilized
Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
浓度：N/A
- 说明书：
Description: Mouse IgG monoclonal antibody for GAA detection. Tested with WB, IHC-P, ICC/IF, FCM in Human.
Background: Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.
