Tempra Carmelo, Scollo Federica, Pannuzzo Martina, Lolicato Fabio, La Rosa Carmelo
Institute of Organic Chemistry and Biochemistry, Prague, Czech Republic.
J. Heyrovský Institute of Physical Chemistry, Czech Academy of Sciences, Prague, Czech Republic.
Biochim Biophys Acta Proteins Proteom. 2022 Apr 1;1870(4):140767. doi: 10.1016/j.bbapap.2022.140767. Epub 2022 Feb 8.
Over the past thirty years, researchers have highlighted the role played by a class of proteins or polypeptides that forms pathogenic amyloid aggregates in vivo, including i) the amyloid Aβ peptide, which is known to form senile plaques in Alzheimer's disease; ii) α-synuclein, responsible for Lewy body formation in Parkinson's disease and iii) IAPP, which is the protein component of type 2 diabetes-associated islet amyloids. These proteins, known as intrinsically disordered proteins (IDPs), are present as highly dynamic conformational ensembles. IDPs can partially (mis) fold into (dys) functional conformations and accumulate as amyloid aggregates upon interaction with other cytosolic partners such as proteins or lipid membranes. In addition, an increasing number of reports link the toxicity of amyloid proteins to their harmful effects on membrane integrity. Still, the molecular mechanism underlying the amyloidogenic proteins transfer from the aqueous environment to the hydrocarbon core of the membrane is poorly understood. This review starts with a historical overview of the toxicity models of amyloidogenic proteins to contextualize the more recent lipid-chaperone hypothesis. Then, we report the early molecular-level events in the aggregation and ion-channel pore formation of Aβ, IAPP, and α-synuclein interacting with model membranes, emphasizing the complexity of these processes due to their different spatial-temporal resolutions. Next, we underline the need for a combined experimental and computational approach, focusing on the strengths and weaknesses of the most commonly used techniques. Finally, the last two chapters highlight the crucial role of lipid-protein complexes as molecular switches among ion-channel-like formation, detergent-like, and fibril formation mechanisms and their implication in fighting amyloidogenic diseases.
在过去三十年中,研究人员强调了一类在体内形成致病性淀粉样聚集体的蛋白质或多肽所起的作用,其中包括:i)淀粉样β肽,已知其在阿尔茨海默病中形成老年斑;ii)α-突触核蛋白,负责帕金森病中路易小体的形成;iii)胰岛淀粉样多肽(IAPP),它是2型糖尿病相关胰岛淀粉样蛋白的蛋白质成分。这些蛋白质被称为内在无序蛋白(IDP),以高度动态的构象集合体形式存在。IDP可以部分(错误)折叠成(功能异常的)构象,并在与其他胞质伴侣(如蛋白质或脂质膜)相互作用时作为淀粉样聚集体积累。此外,越来越多的报告将淀粉样蛋白的毒性与其对膜完整性的有害影响联系起来。然而,淀粉样蛋白从水环境转移到膜的烃核心的分子机制仍知之甚少。本综述首先对淀粉样蛋白毒性模型进行历史概述,以便将最新的脂质伴侣假说置于背景之中。然后,我们报告了Aβ、IAPP和α-突触核蛋白与模型膜相互作用时聚集和离子通道孔形成的早期分子水平事件,强调了由于这些过程不同的时空分辨率而导致的复杂性。接下来,我们强调需要采用实验和计算相结合的方法,重点关注最常用技术的优缺点。最后,最后两章强调了脂质-蛋白质复合物作为离子通道样形成、去污剂样和纤维形成机制之间分子开关的关键作用及其在对抗淀粉样疾病中的意义。