Saarikangas Juha, Caudron Fabrice
a ETH Zurich, Institute of Biochemistry , Zurich , Switzerland.
b Wissenschaftskolleg zu Berlin , Institute for Advanced Study , Berlin , Germany.
Prion. 2017 May 4;11(3):162-173. doi: 10.1080/19336896.2017.1322239. Epub 2017 Jun 2.
Organisms rely on correctly folded proteins to carry out essential functions. Protein quality control factors guard proteostasis and prevent protein misfolding. When quality control fails and in response to diverse stresses, many proteins start to accumulate at specific deposit sites that maintain cellular organization and protect the functionality of coalescing proteins. These transitions involve dedicated proteins that promote coalescence and are facilitated by endo-membranes and cytoskeletal platforms. Moreover, several proteins make use of weak multivalent interactions or conformational templating to drive the formation of large-scale assemblies. Formation of such assemblies is often associated with a change in biochemical activity that can be used by cells to execute biochemical decisions in a localized manner during development and adaption. Since all assembly types impact cell physiology, their localization and dynamics need to be tightly regulated. Interestingly, at least some of the regulatory mechanisms are shared by functional membrane-less organelles and assemblies of terminally aggregated proteins. Furthermore, constituents of functional assemblies can aggregate and become non-functional during aging. Here we present the current knowledge as to how coalescing protein assemblies are spatially organized in cells and we postulate that failures in their spatial confinement might underscore certain aspects of aging and neurodegenerative diseases.
生物体依靠正确折叠的蛋白质来执行基本功能。蛋白质质量控制因子守护蛋白质稳态并防止蛋白质错误折叠。当质量控制失败并响应各种应激时,许多蛋白质开始在特定的沉积位点积累,这些位点维持细胞组织并保护聚集蛋白质的功能。这些转变涉及促进聚集的特定蛋白质,并由内膜和细胞骨架平台促进。此外,几种蛋白质利用弱多价相互作用或构象模板来驱动大规模组装体的形成。这种组装体的形成通常与生化活性的变化相关,细胞可以利用这种变化在发育和适应过程中以局部方式执行生化决策。由于所有组装类型都会影响细胞生理,因此它们的定位和动态需要受到严格调控。有趣的是,至少一些调节机制在功能性无膜细胞器和终末聚集蛋白质的组装体中是共享的。此外,功能性组装体的成分在衰老过程中可能会聚集并变得无功能。在这里,我们介绍了关于聚集蛋白质组装体在细胞中如何进行空间组织的当前知识,并推测它们空间限制的失败可能是衰老和神经退行性疾病某些方面的基础。