Zakharov Stanislav D, Hulleman John D, Dutseva Elena A, Antonenko Yuri N, Rochet Jean-Christophe, Cramer William A
Departments of Biological Sciences, Purdue University, West Lafayette, Indiana 47907-2054, USA.
Biochemistry. 2007 Dec 18;46(50):14369-79. doi: 10.1021/bi701275p. Epub 2007 Nov 22.
Alpha-synuclein (alphaS) is a cytosolic protein involved in the etiology of Parkinson's disease (PD). Disordered in an aqueous environment, alphaS develops a highly helical conformation when bound to membranes having a negatively charged surface and a large curvature. It exhibits a membrane-permeabilizing activity that has been attributed to oligomeric protofibrillar forms. In this study, monomeric wild-type alphaS and two mutants associated with familial PD, E46K and A53T, formed ion channels with well-defined conductance states in membranes containing 25-50% anionic lipid and 50% phosphatidylethanolamine (PE) in the presence of a trans-negative potential. Another familial mutant, A30P, known to have a lower membrane affinity, did not form ion channels. Ca2+ prevented channel formation when added to membranes before alphaS and decreased channel conductance when added to preformed channels. In contrast to the monomer, membrane permeabilization by oligomeric alphaS was not characterized by formation of discrete channels, a requirement for PE lipid, or a membrane potential. Channel activity, alpha-helical content, thermal stability of membrane-bound alphaS determined by far-UV CD, and lateral mobility of alphaS bound to planar membranes measured by fluorescence correlation spectroscopy were correlated. It was inferred that discrete ion channels with well-defined conductance states were formed in the presence of a membrane potential by one or several molecules of monomeric alphaS in an alpha-helical conformation and that such channels may have a role in the normal function and/or pathophysiology of the protein.
α-突触核蛋白(αS)是一种参与帕金森病(PD)病因学的胞质蛋白。在水性环境中无序的αS,当与具有带负电荷表面和大曲率的膜结合时会形成高度螺旋的构象。它表现出一种膜通透活性,这种活性归因于寡聚原纤维形式。在本研究中,单体野生型αS以及与家族性PD相关的两个突变体E46K和A53T,在存在跨膜负电位的情况下,于含有25 - 50%阴离子脂质和50%磷脂酰乙醇胺(PE)的膜中形成了具有明确电导状态的离子通道。另一个家族性突变体A30P,已知其膜亲和力较低,未形成离子通道。在αS加入膜之前添加Ca2 +可阻止通道形成,而在预形成的通道中添加Ca2 +则会降低通道电导。与单体不同,寡聚αS引起的膜通透化并非以离散通道的形成为特征,也不是PE脂质或膜电位的要求。通道活性、α-螺旋含量、通过远紫外圆二色性测定的膜结合αS的热稳定性以及通过荧光相关光谱法测量的与平面膜结合的αS的横向迁移率之间存在相关性。据推断,在膜电位存在的情况下,由一个或几个处于α-螺旋构象的单体αS分子形成了具有明确电导状态的离散离子通道,并且这样的通道可能在该蛋白的正常功能和/或病理生理学中起作用。