La Trobe Institute of Molecular Science La Trobe University Bundoora Victoria Australia.
Howard Florey Institute of Neuroscience and Mental Health Parkville Victoria Australia.
J Extracell Vesicles. 2020 Dec;10(2):e12034. doi: 10.1002/jev2.12034. Epub 2020 Dec 10.
The misfolding and fibrillization of the protein, α-synuclein (αsyn), is associated with neurodegenerative disorders referred to as the synucleinopathies. Understanding the mechanisms of αsyn misfolding is an important area of interest given that αsyn misfolding contributes to disease pathogenesis. While many studies report the ability of synthetic lipid membranes to modulate αsyn folding, there is little data pertaining to the mechanism(s) of this interaction. αSyn has previously been shown to associate with small lipid vesicles released by cells called extracellular vesicles (EVs) and it is postulated these interactions may assist in the spreading of pathological forms of this protein. Together, this presents the need for robust characterisation studies on αsyn fibrillization using biologically-derived vesicles. In this study, we comprehensively characterised the ability of lipid-rich small extracellular vesicles (sEVs) to alter the misfolding of αsyn induced using the Protein Misfolding Cyclic Amplification (PMCA) assay. The biochemical and biophysical properties of misfolded αsyn were examined using a range of techniques including: Thioflavin T fluorescence, transmission electron microscopy, analytical centrifugation and western immunoblot coupled with protease resistance assays and soluble/insoluble fractionation. We show that sEVs cause an acceleration in αsyn fibrillization and provide comprehensive evidence that this results in an increase in the abundance of mature insoluble fibrillar species. In order to elucidate the relevance of the lipid membrane to this interaction, sEV lipid membranes were modified by treatment with methanol, or a combination of methanol and sarkosyl. These treatments altered the ultrastructure of the sEVs without changing the protein cargo. Critically, these modified sEVs had a reduced ability to influence αsyn fibrillization compared to untreated counterparts. This study reports the first comprehensive examination of αsyn:EV interactions and demonstrates that sEVs are powerful modulators of αsyn fibrillization, which is mediated by the sEV membrane. In doing so, this work provides strong evidence for a role of sEVs in contributing directly to αsyn misfolding in the synucleinopathy disorders.
蛋白质 α-突触核蛋白(αsyn)的错误折叠和纤维化与被称为突触核蛋白病的神经退行性疾病有关。鉴于 αsyn 的错误折叠导致疾病发病机制,了解 αsyn 错误折叠的机制是一个重要的研究领域。虽然许多研究报告了合成脂质膜调节 αsyn 折叠的能力,但关于这种相互作用的机制的数据很少。αsyn 先前已被证明与细胞释放的称为细胞外囊泡(EVs)的小脂质囊泡结合,据推测这些相互作用可能有助于这种蛋白质的病理形式的传播。总的来说,这就需要使用来源于生物的囊泡对 αsyn 纤维化进行强有力的特征描述研究。在这项研究中,我们全面研究了富含脂质的小细胞外囊泡(sEVs)通过使用蛋白质错误折叠循环扩增(PMCA)测定改变诱导的 αsyn 错误折叠的能力。使用一系列技术,包括:硫黄素 T 荧光、透射电子显微镜、分析离心和与蛋白酶抗性测定和可溶性/不溶性分级分离相结合的 Western 免疫印迹,研究了错误折叠的 αsyn 的生化和生物物理特性。我们表明 sEVs 导致 αsyn 纤维化加速,并提供了全面的证据表明,这导致成熟的不溶性纤维状物质的丰度增加。为了阐明脂质膜与这种相互作用的相关性,通过用甲醇或甲醇和 Sarkosyl 的混合物处理来修饰 sEV 脂质膜。这些处理改变了 sEV 的超微结构而不改变蛋白质货物。至关重要的是,与未处理的 sEV 相比,这些修饰的 sEV 对 αsyn 纤维化的影响能力降低。这项研究报告了对 αsyn:EV 相互作用的首次全面检查,并表明 sEVs 是 αsyn 纤维化的强大调节剂,这是由 sEV 膜介导的。通过这样做,这项工作为 sEV 直接参与突触核蛋白病中 αsyn 错误折叠提供了有力证据。