Department of Neurosciences, University of California, San Diego/La Jolla, CA, USA, USA.
Trends Neurosci. 2011 Nov;34(11):581-90. doi: 10.1016/j.tins.2011.08.003. Epub 2011 Sep 29.
Multiple system atrophy (MSA) is a neurodegenerative disease involving motor abnormalities that include akinesia, rigidity and postural instability. While improved diagnostic criteria have aided the accurate diagnosis of MSA, our understanding of the neuropathological aspects underlying MSA was bolstered by the identification of α-synuclein (α-syn) as the primary constituent of the abnormal protein aggregates observed in the brains of MSA patients. The generation of transgenic animal models of MSA coupled with an increasing understanding of the biochemical structure and function of α-syn has highlighted a number of key pathological pathways thought to underlie the neurodegeneration observed in MSA. This review summarizes key findings in the field, discusses current areas of debate, and describes current experimental approaches towards disease-modifying therapies.
多系统萎缩（MSA）是一种涉及运动异常的神经退行性疾病，包括运动迟缓、僵硬和姿势不稳。虽然改进的诊断标准有助于准确诊断 MSA，但我们对 MSA 患者大脑中观察到的异常蛋白聚集体的神经病理学基础的理解，得益于 α-突触核蛋白（α-syn）被确定为主要成分。MSA 的转基因动物模型的产生，加上对 α-syn 的生化结构和功能的理解不断增加，突出了许多被认为是 MSA 神经退行性变的关键病理途径。本综述总结了该领域的关键发现，讨论了当前的争议领域，并描述了目前针对疾病修饰治疗的实验方法。
