Secco Valentina, Tiago Tatiana, Staats Roxine, Preet Swapan, Chia Sean, Vendruscolo Michele, Carra Serena
Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, Modena, Italy.
Centre for Misfolding Diseases, Yusuf Hamied Department of Chemistry, University of Cambridge, Cambridge, UK.
iScience. 2024 Aug 3;27(9):110657. doi: 10.1016/j.isci.2024.110657. eCollection 2024 Sep 20.
The process of protein misfolding and aggregation is associated with various cytotoxic effects. Understanding how this phenomenon is regulated by the protein homeostasis system, however, is difficult, since it takes place through a complex non-linear network of coupled microscopic steps, including primary nucleation, fibril elongation, and secondary nucleation, which depend on environmental factors. To address this problem, we studied how the aggregation of α-synuclein, a protein associated with Parkinson's disease, is modulated by molecular chaperones and lipid membranes. We focused on small heat shock proteins (sHSPs/HSPBs), which interact with proteins and lipids and are upregulated during aging, a major risk factor for protein misfolding diseases. HSPBs act on different microscopic steps to prevent α-synuclein aggregation, with HSPB6 showing a lipid-dependent chaperone activity. Our findings provide an example of how HSPBs diversified their mechanisms of action to reach an efficient regulation of protein misfolding and aggregation within the complex cellular environment.
蛋白质错误折叠和聚集的过程与多种细胞毒性效应相关。然而,要理解这种现象是如何被蛋白质稳态系统调控的却很困难,因为它是通过一个复杂的耦合微观步骤的非线性网络发生的,这些步骤包括初级成核、纤维伸长和次级成核,而这些都依赖于环境因素。为了解决这个问题,我们研究了与帕金森病相关的蛋白质α-突触核蛋白的聚集是如何被分子伴侣和脂质膜调节的。我们聚焦于小热休克蛋白(sHSPs/HSPBs),它们与蛋白质和脂质相互作用,并且在衰老过程中上调,而衰老是蛋白质错误折叠疾病的一个主要风险因素。HSPBs作用于不同的微观步骤以防止α-突触核蛋白聚集,其中HSPB6表现出脂质依赖性分子伴侣活性。我们的研究结果提供了一个例子,说明HSPBs如何使它们的作用机制多样化,从而在复杂的细胞环境中实现对蛋白质错误折叠和聚集的有效调控。