The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis
The VCP/p97 system at a glance: connecting cellular function to disease pathogenesis
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DOI：
10.1242/jcs.093831
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发表时间：
2014-09-15
影响因子：
4
通讯作者：
Weihl, Conrad C.
中科院分区：
文献类型：
--
作者：
Meyer, Hemmo;Weihl, Conrad C.
atp酶含缬素蛋白(VCP)/p97已成为泛素系统的核心和重要元素。与辅助因子网络一起，它调节着一个不断扩大的过程范围，几乎延伸到细胞生理学的各个方面。它在蛋白质抑制中的主要作用和在信号通路中的关键功能与退行性疾病和基因组稳定性有关。在这篇细胞科学概览和随附的海报中，我们简要概述了这个复杂的系统。此外，我们讨论了VCP/p97相关疾病的致病基础，然后更详细地强调了与翻译应激反应和RNA生物学有关的令人兴奋的新联系，进一步强调了VCP/p97系统的重要性。
The ATPase valosin-containing protein (VCP)/p97 has emerged as a central and important element of the ubiquitin system. Together with a network of cofactors, it regulates an ever-expanding range of processes that stretch into almost every aspect of cellular physiology. Its main role in proteostasis and key functions in signaling pathways are of relevance to degenerative diseases and genomic stability. In this Cell Science at a Glance and the accompanying poster, we give a brief overview of this complex system. In addition, we discuss the pathogenic basis for VCP/p97-associated diseases and then highlight in more detail new exciting links to the translational stress response and RNA biology that further underscore the significance of the VCP/p97 system.