Department of Medical Biotechnology and Translational Medicine, University of Milano, 20054 Segrate, Milano, Italy.
UK Dementia Research Institute at UCL, London, UK.
Biochim Biophys Acta Mol Cell Biol Lipids. 2023 Sep;1868(9):159350. doi: 10.1016/j.bbalip.2023.159350. Epub 2023 Jun 16.
Fibrillary aggregated α-synuclein represents the neurologic hallmark of Parkinson's disease and is considered to play a causative role in the disease. Although the causes leading to α-synuclein aggregation are not clear, the GM1 ganglioside interaction is recognized to prevent this process. How GM1 exerts these functions is not completely clear, although a primary role of its soluble oligosaccharide (GM1-OS) is emerging. Indeed, we recently identified GM1-OS as the bioactive moiety responsible for GM1 neurotrophic and neuroprotective properties, specifically reverting the parkinsonian phenotype both in in vitro and in vivo models. Here, we report on GM1-OS efficacy against the α-synuclein aggregation and toxicity in vitro. By amyloid seeding aggregation assay and NMR spectroscopy, we demonstrated that GM1-OS was able to prevent both the spontaneous and the prion-like α-synuclein aggregation. Additionally, circular dichroism spectroscopy of recombinant monomeric α-synuclein showed that GM1-OS did not induce any change in α-synuclein secondary structure. Importantly, GM1-OS significantly increased neuronal survival and preserved neurite networks of dopaminergic neurons affected by α-synuclein oligomers, together with a reduction of microglia activation. These data further demonstrate that the ganglioside GM1 acts through its oligosaccharide also in preventing the α-synuclein pathogenic aggregation in Parkinson's disease, opening a perspective window for GM1-OS as drug candidate.
纤维状聚集的α-突触核蛋白是帕金森病的神经学标志,被认为在疾病中起因果作用。尽管导致α-突触核蛋白聚集的原因尚不清楚,但已认识到 GM1 神经节苷脂相互作用可防止这一过程。GM1 如何发挥这些功能尚不完全清楚,尽管其可溶性寡糖(GM1-OS)的主要作用正在显现。事实上,我们最近确定 GM1-OS 是负责 GM1 神经营养和神经保护特性的生物活性部分,特别是在体外和体内模型中逆转帕金森病表型。在这里,我们报告了 GM1-OS 对α-突触核蛋白聚集和体外毒性的疗效。通过淀粉样蛋白种子聚合测定和 NMR 光谱,我们证明 GM1-OS 能够预防自发和类朊病毒样α-突触核蛋白聚集。此外,重组单体α-突触核蛋白的圆二色性光谱表明 GM1-OS 不会诱导α-突触核蛋白二级结构发生任何变化。重要的是,GM1-OS 显著增加了受α-突触核蛋白寡聚物影响的多巴胺能神经元的存活并维持了其神经元网络,同时减少了小胶质细胞的激活。这些数据进一步表明,神经节苷脂 GM1 通过其寡糖也可预防帕金森病中α-突触核蛋白的致病聚集,为 GM1-OS 作为候选药物开辟了一个新的视角。