GAA 抗体 (N-Term)
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Quick Overview for GAA 抗体 (N-Term) (ABIN656475)
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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抗原表位
- AA 174-203, N-Term
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纯化方法
- This antibody is purified through a protein A column, followed by peptide affinity purification.
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免疫原
- This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.
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亚型
- IgG
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应用备注
- WB: 1:2000. WB: 1:1000. WB: 1:1000. WB: 1:1000. IHC-P-Leica: 1:500. IHC-P-Leica: 1:500
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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
- Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) 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
- 4 °C,-20 °C
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储存方法
- GAA Antibody (N-term) can be refrigerated at 2-8 °C for up to 6 months. For long term storage, place the at -20 °C.
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有效期
- 6 months
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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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分子量
- 105324
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基因ID
- 2548
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NCBI登录号
- NP_000143, NP_001073271, NP_001073272
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UniProt
- P10253
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途径
- Cellular Glucan Metabolic Process
抗原
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