GAA 抗体 (AA 494-527)
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Quick Overview for GAA 抗体 (AA 494-527) (ABIN5647392)
抗原
See all GAA 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 494-527
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纯化方法
- Antigen affinity purified
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免疫原
- Amino acids 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody.
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亚型
- IgG
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应用备注
- Optimal dilution of the GAA antibody should be determined by the researcher.\. Western blot: 0.5-1 μg/mL,Immunohistochemistry (FFPE): 1-2 μg/mL
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限制
- 仅限研究用
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缓冲液
- 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water
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储存条件
- -20 °C
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储存方法
- After reconstitution, the GAA antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.
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- GAA (Glucosidase, Alpha, Acid (GAA))
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别名
- GAA / Glucosidase alpha acid
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背景
- 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.
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UniProt
- P10253
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途径
- Cellular Glucan Metabolic Process
抗原
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