Breydo Leonid, Wu Jessica W, Uversky Vladimir N
Department of Molecular Medicine, College of Medicine, University of South Florida, Tampa, FL 33612, USA.
Biochim Biophys Acta. 2012 Feb;1822(2):261-85. doi: 10.1016/j.bbadis.2011.10.002. Epub 2011 Oct 12.
Substantial evidence links α-synuclein, a small highly conserved presynaptic protein with unknown function, to both familial and sporadic Parkinson's disease (PD). α-Synuclein has been identified as the major component of Lewy bodies and Lewy neurites, the characteristic proteinaceous deposits that are the hallmarks of PD. α-Synuclein is a typical intrinsically disordered protein, but can adopt a number of different conformational states depending on conditions and cofactors. These include the helical membrane-bound form, a partially-folded state that is a key intermediate in aggregation and fibrillation, various oligomeric species, and fibrillar and amorphous aggregates. The molecular basis of PD appears to be tightly coupled to the aggregation of α-synuclein and the factors that affect its conformation. This review examines the different aggregation states of α-synuclein, the molecular mechanism of its aggregation, and the influence of environmental and genetic factors on this process.
大量证据表明,α-突触核蛋白(一种功能未知的高度保守的小突触前蛋白)与家族性和散发性帕金森病(PD)均有关联。α-突触核蛋白已被确定为路易小体和路易神经突的主要成分,这些特征性蛋白质沉积物是帕金森病的标志。α-突触核蛋白是一种典型的内在无序蛋白,但根据条件和辅助因子的不同,它可以呈现多种不同的构象状态。这些状态包括螺旋膜结合形式、部分折叠状态(这是聚集和纤维化过程中的关键中间体)、各种寡聚体以及纤维状和无定形聚集体。帕金森病的分子基础似乎与α-突触核蛋白的聚集以及影响其构象的因素紧密相关。本综述探讨了α-突触核蛋白的不同聚集状态、其聚集的分子机制以及环境和遗传因素对这一过程的影响。