Progerin 蛋白
This Recombinant Progerin protein is expressed in 大肠杆菌（E. Coli）.
产品编号 ABIN2669340
发货至:
中国
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Quick Overview for Progerin 蛋白 (ABIN2669340)
抗原
Progerin
蛋白类型
Recombinant
宿主
人
资源
大肠杆菌（E. Coli）
应用范围
SDS-PAGE (SDS), Western Blotting (WB)
纯度
> 90 %
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应用备注
- Optimal working dilution should be determined by the investigator.
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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
- 20 mM Tris-HCl pH 7.5, 0,5M NaCl, 1 mM DTT, 1,5 mM EDTA, 20 % (v/v) glycerol
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储存条件
- -20 °C
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- Progerin
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背景
- Progerin is a 614 amino acids protein involved in Hutchinson-Gilford progeria syndrome. Progerin is most often generated by a point mutation (C1824T) in the LMNA gene that codes for lamin A and C. This mutation activates a cryptic splice site and gives rise to a form of lamin A with a 50-amino acids internal deletion within the carboxyl-terminal domain of the protein. Approximately 80 % of Hutchinson-Gilford progeria syndrome cases carry a single copy of the most common mutation, a silent point mutation, G608G (GGC > GGT), within exon 11 of LMNA gene. Lamin A constitutes a major structural component of the lamina, a scaffold of proteins found inside the nuclear membrane of a cell, progerin does not properly integrate into the lamina, which disrupts the scaffold structure and leads to significant disfigurement of the nucleus, characterized by a lobular shape.
抗原
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