GAA 抗体
Quick Overview for GAA 抗体 (ABIN6989688)
抗原
See all GAA 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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交叉反应
- 人
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纯化方法
- Purified by Protein A.
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免疫原
- Synthetic peptide within Human GAA.
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亚型
- IgG
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应用备注
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WB 1:300-5000
FCM 1:20-100
IHC-P 1:200-400 -
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 1xTBS ( pH 7.4), 1 % BSA, 40 %Glycerol and 0.05 % Sodium Azide.
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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 for 12 months.
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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 antibody, Acid alpha glucosidase antibody, Acid maltase antibody, Aglucosidase alfa antibody, Alpha glucosidase antibody, GAA antibody, Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody, Glucosidase alpha acid antibody, Glucosidase alpha antibody, LYAG antibody, LYAG_HUMAN antibody, Lysosomal alpha glucosidase antibody
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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