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在Situ架构和细胞交互的PolyQ包含
Felix J B Bäuerlein1, Itika Saha2, Archana Mishra3
1Department of Molecular Structural Biology, Max Planck Institute of Biochemistry, 82152 Martinsried, Germany.
Cell
|September 12, 2017
概括
细胞中的蛋白质聚合物形成粉样纤维,损伤细胞内膜,特别是细胞内膜 (ER). 这种相互作用会损害ER的组织和动态,导致蛋白质聚合疾病的细胞功能障碍.
科学领域:
- 细胞生物学
- 神经科学
- 生物化学
背景情况:
- 与疾病相关的蛋白质可以形成细胞毒性细胞内包容体.
- 了解蛋白质聚合物的局部结构和细胞作用对于疾病病理学的洞察至关重要.
研究的目的:
- 调查多重胺 (polyQ) 扩展的亨廷丁外子1的局部结构.
- 分析这些蛋白质聚合物与细胞内膜之间的相互作用.
主要方法:
- 使用先进的冷电子断层扫描检查完整细胞内的入.
- 主要的小鼠神经元和永生的人类细胞被用作模型系统.
主要成果:
- 聚含有与细胞内膜相互作用的粉样纤维,特别是细胞内膜 (ER).
- 这些纤维会导致膜变形,破坏ER组织,并改变包容外围的ER膜动态.
结论:
- 粉状纤维和内膜之间的异常相互作用有助于蛋白质聚合引起的细胞损伤.
- 这项研究提供了蛋白质聚合物如何影响细胞器官的结构见解,提供了潜在的治疗点.
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