Yang Yunying, Chen Sichun, Gao Yan, Huang Liqin, Liu Zehua, Liu Chaoyang, Fang Xin, Zhang Zhentao
Department of Neurology, Renmin Hospital of Wuhan University, Wuhan, 430060, China.
Research Center for Environment and Health, Zhongnan University of Economics and Law, Wuhan, 430073, China.
Mol Neurobiol. 2025 Apr;62(4):4605-4616. doi: 10.1007/s12035-024-04561-2. Epub 2024 Oct 29.
The deposition of pathological α-synuclein (α-Syn) in the central nervous system (CNS) is a hallmark of Parkinson's disease (PD). Notably, pathological α-Syn exists not only in the CNS but also in peripheral organs and body fluids in PD patients. Emerging evidence has shown the transmission of α-Syn pathology from the body to the brain. Nevertheless, the factors that drive the aggregation of peripheral α-Syn remain largely unknown. Here, we revealed that complement receptor 1 (CR1), a component of the peripheral blood system, acts as a promoter of α-Syn pathology. The transmembrane domain of CR1 (CR1-TM) exacerbates α-Syn phosphorylation and aggregation in vitro. Furthermore, intravenous injection of α-Syn fibrils induced the formation of α-Syn pathology in the brain. Co-administration of CR1-TM exacerbated α-Syn pathology induced by intravenous injection of preformed α-Syn fibrils. Our findings suggest that extracerebral factors such as CR1 can drive α-Syn pathology and serve as therapeutic targets for treating synucleinopathies.
病理性α-突触核蛋白(α-Syn)在中枢神经系统(CNS)中的沉积是帕金森病(PD)的一个标志。值得注意的是,病理性α-Syn不仅存在于CNS中,还存在于PD患者的外周器官和体液中。新出现的证据表明α-Syn病理学可从身体传播至大脑。然而,驱动外周α-Syn聚集的因素在很大程度上仍不清楚。在此,我们揭示了外周血液系统的一个成分补体受体1(CR1)是α-Syn病理学的一个促进因子。CR1的跨膜结构域(CR1-TM)在体外加剧α-Syn的磷酸化和聚集。此外,静脉注射α-Syn原纤维可诱导大脑中α-Syn病理学的形成。共同给予CR1-TM会加剧静脉注射预先形成的α-Syn原纤维所诱导的α-Syn病理学。我们的研究结果表明,诸如CR1等脑外因素可驱动α-Syn病理学,并可作为治疗突触核蛋白病的治疗靶点。