GAA 抗体 (AA 174-203)
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Quick Overview for GAA 抗体 (AA 174-203) (ABIN7871285)
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 174-203
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原理
- GAA Antibody / Glucosidase alpha acid
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纯化方法
- Antigen affinity purified
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免疫原
- A portion of amino acids 174-203 from the human protein were used as the immunogen for the GAA antibody.
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亚型
- Ig Fraction
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应用备注
- The stated application concentrations are suggested starting points. Titration of the GAA antibody may be required due to differences in protocols and secondary/substrate sensitivity.
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- In 1X PBS, pH 7.4, with 0.09 % sodium azide
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Aliquot the GAA antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
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- GAA (Glucosidase, Alpha, Acid (GAA))
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别名
- GAA
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背景
- 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.
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UniProt
- P10253
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途径
- Cellular Glucan Metabolic Process
抗原
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