Türkisch-Deutsche Universität, Theoretical and Computational Biophysics Group, Molecular Biotechnology, Sahinkaya Caddesi, No. 86, Beykoz, Istanbul 34820, Turkey.
Department of Molecular Medicine and USF Health Byrd Alzheimer's Research Institute, Morsani College of Medicine, University of South Florida, Tampa, FL 33612, USA.
Int J Mol Sci. 2018 Jan 24;19(2):336. doi: 10.3390/ijms19020336.
Amyloid-β and α-synuclein are intrinsically disordered proteins (IDPs), which are at the center of Alzheimer's and Parkinson's disease pathologies, respectively. These IDPs are extremely flexible and do not adopt stable structures. Furthermore, both amyloid-β and α-synuclein can form toxic oligomers, amyloid fibrils and other type of aggregates in Alzheimer's and Parkinson's diseases. Experimentalists face challenges in investigating the structures and thermodynamic properties of these IDPs in their monomeric and oligomeric forms due to the rapid conformational changes, fast aggregation processes and strong solvent effects. Classical molecular dynamics simulations complement experiments and provide structural information at the atomic level with dynamics without facing the same experimental limitations. Artificial missense mutations are employed experimentally and computationally for providing insights into the structure-function relationships of amyloid-β and α-synuclein in relation to the pathologies of Alzheimer's and Parkinson's diseases. Furthermore, there are several natural genetic variations that play a role in the pathogenesis of familial cases of Alzheimer's and Parkinson's diseases, which are related to specific genetic defects inherited in dominant or recessive patterns. The present review summarizes the current understanding of monomeric and oligomeric forms of amyloid-β and α-synuclein, as well as the impacts of artificial and pathological missense mutations on the structural ensembles of these IDPs using molecular dynamics simulations. We also emphasize the recent investigations on residual secondary structure formation in dynamic conformational ensembles of amyloid-β and α-synuclein, such as β-structure linked to the oligomerization and fibrillation mechanisms related to the pathologies of Alzheimer's and Parkinson's diseases. This information represents an important foundation for the successful and efficient drug design studies.
淀粉样蛋白-β和α-突触核蛋白是两种无序蛋白(IDPs),分别是阿尔茨海默病和帕金森病病理的核心。这两种 IDP 非常灵活,不采用稳定的结构。此外,淀粉样蛋白-β和α-突触核蛋白在阿尔茨海默病和帕金森病中都可以形成毒性寡聚体、淀粉样纤维和其他类型的聚集体。由于快速构象变化、快速聚集过程和强溶剂效应,实验人员在研究这些 IDP 的单体和寡聚体形式的结构和热力学性质时面临挑战。经典的分子动力学模拟补充了实验,可以在不面临相同实验限制的情况下提供原子水平的结构信息和动力学信息。人工错义突变在实验和计算中被用来深入了解淀粉样蛋白-β和α-突触核蛋白与阿尔茨海默病和帕金森病的病理关系的结构-功能关系。此外,还有几种自然遗传变异在家族性阿尔茨海默病和帕金森病的发病机制中起作用,这些变异与显性或隐性遗传模式下的特定遗传缺陷有关。本综述总结了目前对淀粉样蛋白-β和α-突触核蛋白单体和寡聚体形式的理解,以及使用分子动力学模拟研究人工和病理性错义突变对这些 IDP 结构集合的影响。我们还强调了最近对淀粉样蛋白-β和α-突触核蛋白动态构象集合中残留二级结构形成的研究,如与寡聚化和纤维化机制相关的β-结构与阿尔茨海默病和帕金森病的病理有关。这些信息为成功和有效的药物设计研究提供了重要的基础。