多系统萎缩的神经病理学及其治疗意义。
The neuropathology of multiple system atrophy and its therapeutic implications.
作者信息
Valera Elvira, Masliah Eliezer
机构信息
Department of Neurosciences, University of California, San Diego, 9500 Gilman Dr, La Jolla, CA 92093, USA.
Division of Neurosciences, National Institute on Aging/NIH, 7201 Wisconsin Ave, Bethesda, MD 20814, USA.
出版信息
Auton Neurosci. 2018 May;211:1-6. doi: 10.1016/j.autneu.2017.11.002. Epub 2017 Nov 10.
Multiple system atrophy (MSA) is a fatal neurodegenerative disorder characterized by the abnormal accumulation of toxic forms of the synaptic protein alpha-synuclein (α-syn) within oligodendrocytes and neurons. The presence of α-syn within oligodendrocytes in the form of glial cytoplasmic inclusions is the diagnostic hallmark of MSA. However, it has been postulated that α-syn is produced in neurons and propagates to oligodendrocytes, where unknown mechanisms lead to its accumulation. The presence of α-syn within neurons in MSA has not been so extensively studied, but it may shed light into neuropathological mechanisms leading to oligodendroglial accumulation. Here we summarize the principal neuropathological events of MSA, and discuss how a deeper knowledge of these mechanisms may help develop effective therapies targeting α-syn accumulation and spreading.
多系统萎缩（MSA）是一种致命的神经退行性疾病，其特征是突触蛋白α-突触核蛋白（α-syn）的毒性形式在少突胶质细胞和神经元内异常积聚。少突胶质细胞内以胶质细胞质内含物形式存在的α-syn是MSA的诊断标志。然而，据推测α-syn在神经元中产生并传播到少突胶质细胞，在少突胶质细胞中未知机制导致其积聚。MSA中神经元内α-syn的存在尚未得到如此广泛的研究，但它可能有助于揭示导致少突胶质细胞积聚的神经病理机制。在这里，我们总结了MSA的主要神经病理事件，并讨论了对这些机制的更深入了解如何有助于开发针对α-syn积聚和扩散的有效疗法。
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