Yang Ze-Xian, Zhang Yu, Wang Qing, Zhang Lei, Liu Yi-Fei, Zhang Ye, Ren Yu, Zhou Chen, Gao Hui-Wen, Zhang Nai-Xia, Feng Lin-Yin
CAS Key Laboratory of Receptor Research, Center for Neurological and Psychiatric Research and Drug Discovery (CNPRDD), Shanghai Institute of Materia Medica, Chinese Academy of Sciences, 555 Zu Chong Zhi Road, Shanghai, 201203, China.
University of Chinese Academy of Sciences, No. 19A Yuquan Road, Beijing, 100049, China.
Acta Pharmacol Sin. 2024 Jan;45(1):36-51. doi: 10.1038/s41401-023-01150-2. Epub 2023 Sep 8.
The gut-brain axis plays a vital role in Parkinson's disease (PD). The mechanisms of gut-brain transmission mainly focus on α-synuclein deposition, intestinal inflammation and microbiota function. A few studies have shown the trigger of PD pathology in the gut. α-Synuclein is highly conserved in food products, which was able to form β-folded aggregates and to infect the intestinal mucosa. In this study we investigated whether α-synuclein-preformed fibril (PFF) exposure could modulate the intestinal environment and induce rodent models replicating PD pathology. We first showed that PFF could be internalized into co-cultured Caco-2/HT29/Raji b cells in vitro. Furthermore, we demonstrated that PFF perfusion caused the intestinal inflammation and activation of enteric glial cells in an ex vivo intestinal organ culture and in an in vivo intestinal mouse coloclysis model. Moreover, we found that PFF exposure through regular coloclysis induced PD pathology in wild-type (WT) and A53T α-synuclein transgenic mice with various phenotypes. Particularly in A53T mice, PFF induced significant behavioral disorders, intestinal inflammation, α-synuclein deposition, microbiota dysbiosis, glial activation as well as degeneration of dopaminergic neurons in the substantia nigra. In WT mice, however, the PFF induced only mild behavioral abnormalities, intestinal inflammation, α-synuclein deposition, and glial activation, without significant changes in microbiota and dopaminergic neurons. Our results reveal the possibility of α-synuclein aggregates binding to the intestinal mucosa and modeling PD in mice. This study may shed light on the investigation and early intervention of the gut-origin hypothesis in neurodegenerative diseases.
肠-脑轴在帕金森病(PD)中起着至关重要的作用。肠-脑传递机制主要集中在α-突触核蛋白沉积、肠道炎症和微生物群功能上。一些研究已经表明肠道中帕金森病病理的触发因素。α-突触核蛋白在食品中高度保守,能够形成β折叠聚集体并感染肠黏膜。在本研究中,我们调查了α-突触核蛋白预形成纤维(PFF)暴露是否能调节肠道环境并诱导复制帕金森病病理的啮齿动物模型。我们首先表明PFF在体外可被共培养的Caco-2/HT29/Raji b细胞内化。此外,我们证明PFF灌注在离体肠道器官培养和体内肠道小鼠灌肠模型中引起肠道炎症和肠胶质细胞激活。而且,我们发现通过定期灌肠暴露PFF可在具有不同表型的野生型(WT)和A53Tα-突触核蛋白转基因小鼠中诱导帕金森病病理。特别是在A53T小鼠中,PFF诱导了显著的行为障碍、肠道炎症、α-突触核蛋白沉积、微生物群失调、胶质细胞激活以及黑质中多巴胺能神经元的退化。然而,在WT小鼠中,PFF仅诱导了轻微的行为异常、肠道炎症、α-突触核蛋白沉积和胶质细胞激活,微生物群和多巴胺能神经元没有显著变化。我们的结果揭示了α-突触核蛋白聚集体与肠黏膜结合并在小鼠中模拟帕金森病的可能性。本研究可能为神经退行性疾病中肠道起源假说的研究和早期干预提供线索。