GAA 抗体 (N-Term)
Quick Overview for GAA 抗体 (N-Term) (ABIN2797807)
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, N-Term
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原理
- Rabbit Anti-Human GAA (N-term) Antibody
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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
- Ig Fraction
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应用备注
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Western Blot, Immunohistochemistry
Recommended Dilutions
WB: 1:1000, IHC: 1:10-50GAA Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated. -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 0.460 mg/mL
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储存条件
- 4 °C,-20 °C
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储存方法
- 2-8°C (short-term), -20°C (long-term)
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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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Target Description: 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.
Gene Symbol: GAA
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分子量
- 105324 Da
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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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