GAA 抗体 (AA 432-482)
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Quick Overview for GAA 抗体 (AA 432-482) (ABIN8121679)
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 432-482
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原理
- LYAG Antibody
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特异性
- This antibody detects endogenous levels of LYAG at Human/Mouse/Rat
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纯化方法
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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免疫原
- Synthesized peptide derived from human LYAG AA range: 432-482
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亚型
- IgG
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应用备注
- WB 1:500-2000
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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
- Liquid in PBS containing 50 % glycerol, 0.5 % BSA and 0.02 % 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
- Store at -20°C/1 year
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有效期
- 12 months
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- GAA (Glucosidase, Alpha, Acid (GAA))
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别名
- LYAG
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背景
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Synonyms: Lysosomal alpha-glucosidase (EC 3.2.1.20) (Acid maltase) (Aglucosidase alfa) [Cleaved into: 76 kDa lysosomal alpha-glucosidase; 70 kDa lysosomal alpha-glucosidase]
Background: This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016],
Gene Name: GAA
Protein Name: LYAG
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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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