Liddle Rodger A
Department of Medicine, Duke University Medical Center and Department of Veterans Affairs Health Care System, Durham, NC 27710, United States.
Brain Res. 2018 Aug 15;1693(Pt B):201-206. doi: 10.1016/j.brainres.2018.01.010. Epub 2018 Jan 31.
Parkinson's disease (PD) is a debilitating neurodegenerative condition associated with tremor, rigidity, dementia, and gastrointestinal symptoms such as constipation, nausea and vomiting. The pathological hallmarks of PD are Lewy bodies and neurites in the brain and peripheral nerves. The major constituent of Lewy bodies is the neuronal protein α-synuclein. Misfolding of α-synuclein confers prion-like properties enabling its spread from cell to cell. Misfolded α-synuclein also serves as a template and induces misfolding of endogenous α-synuclein in recipient cells leading to the formation of oligomers that progress to fibrils and eventually Lewy bodies. Accumulating evidence suggests that PD may arise in the gut. Clinically, gastrointestinal symptoms often appear in patients before other neurological signs and aggregates of α-synuclein have been found in enteric nerves of PD patients. Importantly, patients undergoing vagotomy have a reduced risk of developing PD. Experimentally, abnormal forms of α-synuclein appear in enteric nerves before they appear in the brain and injection of abnormal α-synuclein into the wall of the intestine spreads to the vagus nerve. Ingested toxins and alterations in gut microbiota can induce α-synuclein aggregation and PD, however, it is not known how PD starts. Recently, it has been shown that sensory cells of the gut known as enteroendocrine cells (EECs) contain α-synuclein and synapse with enteric nerves, thus providing a connection from the gut to the brain. It is possible that abnormal α-synuclein first develops in EECs and spreads to the nervous system.
帕金森病(PD)是一种使人衰弱的神经退行性疾病,与震颤、僵硬、痴呆以及便秘、恶心和呕吐等胃肠道症状有关。PD的病理特征是大脑和周围神经中的路易小体和神经突。路易小体的主要成分是神经元蛋白α-突触核蛋白。α-突触核蛋白的错误折叠赋予其朊病毒样特性,使其能够在细胞间传播。错误折叠的α-突触核蛋白还作为模板,诱导受体细胞内源性α-突触核蛋白的错误折叠,导致形成寡聚体,进而发展为纤维,最终形成路易小体。越来越多的证据表明,PD可能起源于肠道。临床上,胃肠道症状通常在患者出现其他神经体征之前出现,并且在PD患者的肠神经中发现了α-突触核蛋白聚集体。重要的是,接受迷走神经切断术的患者患PD的风险降低。在实验中,α-突触核蛋白的异常形式在出现在大脑之前先出现在肠神经中,并且将异常的α-突触核蛋白注射到肠壁会扩散到迷走神经。摄入的毒素和肠道微生物群的改变可诱导α-突触核蛋白聚集和PD,然而,PD如何开始尚不清楚。最近,研究表明,被称为肠内分泌细胞(EECs)的肠道感觉细胞含有α-突触核蛋白,并与肠神经形成突触,从而提供了从肠道到大脑的连接。异常的α-突触核蛋白可能首先在EECs中形成并扩散到神经系统。