GAA 抗体 (AA 541-640) (Cy7)
Quick Overview for GAA 抗体 (AA 541-640) (Cy7) (ABIN1708509)
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 541-640
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交叉反应
- 人, 小鼠, 大鼠
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预测反应
- Dog,Sheep,Pig,Horse
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha
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亚型
- IgG
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应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: 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
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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有效期
- 12 months
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- GAA (Glucosidase, Alpha, Acid (GAA))
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别名
- GAA
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背景
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Synonyms: 70 kDa lysosomal alpha-glucosidase, Acid alpha glucosidase, Acid maltase, Aglucosidase alfa, Alpha glucosidase, GAA, Glucosidase alpha acid Pompe disease glycogen storage disease type II, Glucosidase alpha acid, Glucosidase alpha, LYAG, LYAG_HUMAN, Lysosomal alpha glucosidase.
Background: 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. [provided by RefSeq, Jul 2008].
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基因ID
- 2548
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UniProt
- P10253
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途径
- Cellular Glucan Metabolic Process
抗原
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