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与ALS相关的VapB P56S突变改变了突触中的神经元线粒体周转率
Hiu-Tung C Wong1, Angelica E Lang1,2, Chris Stein1
1Department of Integrative Biology, University of Wisconsin-Madison, Madison, Wisconsin 53706.
概括
囊泡相关膜蛋白相关蛋白B (VapB) 调节神经元中的线粒体循环. 一个与ALS相关的突变会损害线粒,导致线粒体数量增加和潜在的神经退行.
科学领域:
- 神经科学是一个神经科学.
- 细胞生物学 细胞生物学
- 遗传学 遗传学是一种遗传学.
背景情况:
- 线粒体平衡对于神经元功能和生存至关重要.
- 细胞内膜网膜 (ER) -线粒体接触点调节线粒体动力学.
- 囊泡关联膜蛋白关联蛋白B (VapB) 是这些接触点的ER蛋白,一个突变 (P56S) 与8型肌缩侧硬化症 (ALS) 有关.
研究的目的:
- 研究VapB在神经元线粒体人口维持中的作用.
- 确定VapB P56S突变如何影响线粒体平衡.
- 阐明VapB控制线粒体周转的机制.
主要方法:
- 转基因斑马鱼幼虫表达野生类型的VapB和VapB P56S.
- 基因操纵来改变线粒体生物发生.
- 在轴突终端中评估线粒体生物发生和线粒体细胞衰变.
- 使用一种合成的ER-线粒体.
主要成果:
- 转基因VapB P56S表达增加了线粒体生物发生和轴突终端的种群大小.
- 野生类型的VapB表达增加了线粒,平衡了生物发生.
- VapB P56S突变体表现出 mitoophagy 功能受损,无法弥补生物发生率的增加.
- VapB在线粒体周转中的作用似乎独立于ER-线粒体结合.
结论:
- VapB调节神经元轴突终端中的线粒体周转率.
- 与ALS相关的VapB P56S突变损害了线粒体,破坏了线粒体的人口控制.
- 这种受损的VapB功能可能会导致ALS型8中观察到的神经退行.
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