Nuermaimaiti Maierdanjiang, Ishikawa Kei-Ichi, Oyama Genko, Nonaka Risa, Shiga Takahiro, Jo Takayuki, Tsunemi Taiji, Nakamura Ryota, Krüger Rejko, Akamatsu Wado, Hattori Nobutaka
Department of Neurology, Faculty of Medicine, Juntendo University, Tokyo, Japan; Department of Clinical Data of Parkinson's Disease, Graduate School of Medicine, Juntendo University, Tokyo, Japan.
Department of Neurology, Faculty of Medicine, Juntendo University, Tokyo, Japan; Center for Genomic and Regenerative Medicine, Graduate School of Medicine, Juntendo University, Tokyo, Japan; Department of Research and Development for Organoids, School of Medicine, Juntendo University, Tokyo, Japan.
Neurosci Res. 2025 Mar;212:105-114. doi: 10.1016/j.neures.2024.11.007. Epub 2024 Nov 29.
Lewy body diseases, including Parkinson's disease (PD), are characterized by the spread of alpha-synuclein (αSyn) between neurons across synapses, a process crucial for understanding their pathophysiology and developing effective treatments. In this study, we aimed to investigate the role of neuronal activity in releasing αSyn from human induced pluripotent stem cell-derived dopaminergic neurons. We examined human induced pluripotent stem cell-derived dopaminergic neurons, both healthy and those with the αSyn gene mutation associated with PD. We employed pharmacological agents and optogenetic techniques and demonstrated that increased neuronal activity, induced by bicuculline or optogenetic stimulation, significantly enhances αSyn release. However, suppression of neuronal activity with cyanquixaline reduces αSyn secretion. These findings underscore the pivotal role of neuronal activity in αSyn transmission between neurons, showing its potential impact on the spread of Lewy pathology in patients with neurodegenerative diseases like PD. Therefore, this study advances our understanding of PD and opens new avenues for therapeutic strategies to mitigate Lewy body disease progression.
路易体疾病,包括帕金森病(PD),其特征是α-突触核蛋白(αSyn)在神经元之间通过突触传播,这一过程对于理解其病理生理学和开发有效治疗方法至关重要。在本研究中,我们旨在研究神经元活动在从人诱导多能干细胞衍生的多巴胺能神经元中释放αSyn方面的作用。我们检查了人诱导多能干细胞衍生的多巴胺能神经元,包括健康的以及携带与PD相关的αSyn基因突变的神经元。我们使用了药理学试剂和光遗传学技术,并证明由荷包牡丹碱或光遗传学刺激诱导的神经元活动增加会显著增强αSyn的释放。然而,用氰喹沙林抑制神经元活动会减少αSyn的分泌。这些发现强调了神经元活动在神经元之间αSyn传递中的关键作用,显示了其对像PD这样的神经退行性疾病患者路易病理传播的潜在影响。因此,本研究增进了我们对PD的理解,并为减轻路易体疾病进展的治疗策略开辟了新途径。