From the Laboratory of Protein Conformation and Dynamics, Biochemistry and Biophysics Center, NHLBI, National Institutes of Health, Bethesda, Maryland 20892.
From the Laboratory of Protein Conformation and Dynamics, Biochemistry and Biophysics Center, NHLBI, National Institutes of Health, Bethesda, Maryland 20892
J Biol Chem. 2018 Jan 19;293(3):767-776. doi: 10.1074/jbc.M117.812388. Epub 2017 Nov 30.
Parkinson's disease (PD) is associated with the formation of α-synuclein amyloid fibrils. Elucidating the role of these β-sheet-rich fibrils in disease progression is crucial; however, collecting detailed structural information on amyloids is inherently difficult because of their insoluble, non-crystalline, and polymorphic nature. Here, we show that Raman spectroscopy is a facile technique for characterizing structural features of α-synuclein fibrils. Combining Raman spectroscopy with aggregation kinetics and transmission electron microscopy, we examined the effects of pH and ionic strength as well as four PD-related mutations (A30P, E46K, G51D, and A53T) on α-synuclein fibrils. Raman spectral differences were observed in the amide-I, amide-III, and fingerprint regions, indicating that secondary structure and tertiary contacts are influenced by pH and to a lesser extent by NaCl. Faster aggregation times appear to facilitate unique fibril structure as determined by the highly reproducible amide-I band widths, linking aggregation propensity and fibril polymorphism. Importantly, Raman spectroscopy revealed molecular-level perturbations of fibril conformation by the PD-related mutations that are not apparent through transmission electron microscopy or limited proteolysis. The amide-III band was found to be particularly sensitive, with G51D exhibiting the most distinctive features, followed by A53T and E46K. Relating to a cellular environment, our data would suggest that fibril polymorphs can be formed in different cellular compartments and potentially result in distinct phenotypes. Our work sets a foundation toward future cellular Raman studies of amyloids.
帕金森病(PD)与α-突触核蛋白淀粉样纤维的形成有关。阐明这些富含β-折叠的纤维在疾病进展中的作用至关重要;然而,由于其不溶性、非晶态和多态性,收集有关淀粉样蛋白的详细结构信息具有内在的困难。在这里,我们表明拉曼光谱是一种用于表征α-突触核蛋白纤维结构特征的简便技术。我们结合拉曼光谱、聚集动力学和透射电子显微镜,研究了 pH 值和离子强度以及四种 PD 相关突变(A30P、E46K、G51D 和 A53T)对α-突触核蛋白纤维的影响。在酰胺-I、酰胺-III 和指纹区域观察到拉曼光谱差异,表明二级结构和三级接触受 pH 值影响,受 NaCl 影响较小。更快的聚集时间似乎促进了独特的纤维结构,这可以通过高度可重复的酰胺-I 带宽来确定,将聚集倾向与纤维多态性联系起来。重要的是,拉曼光谱揭示了 PD 相关突变对纤维构象的分子水平扰动,这在透射电子显微镜或有限蛋白酶解中并不明显。发现酰胺-III 带特别敏感,G51D 表现出最独特的特征,其次是 A53T 和 E46K。与细胞环境有关,我们的数据表明,纤维多态体可以在不同的细胞隔室中形成,并可能导致不同的表型。我们的工作为未来对细胞内淀粉样蛋白的拉曼研究奠定了基础。