Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA.
Neuroscience Graduate Program, University of Michigan, Ann Arbor, MI 48109, USA.
Cells. 2023 Feb 13;12(4):601. doi: 10.3390/cells12040601.
Human studies, in combination with animal and cellular models, support glial cells as both major contributors to neurodegenerative diseases and promising therapeutic targets. Among glial cells, oligodendrocytes and Schwann cells are the myelinating glial cells of the central and peripheral nervous system, respectively. In this review, we discuss the contributions of these central and peripheral myelinating glia to the pathomechanisms of polyglutamine (polyQ) spinocerebellar ataxia (SCA) types 1, 2, 3, 6, 7, and 17. First, we highlight the function of oligodendrocytes in healthy conditions and how they are disrupted in polyQ SCA patients and diseased model systems. We then cover the role of Schwann cells in peripheral nerve function and repair as well as their possible role in peripheral neuropathy in polyQ SCAs. Finally, we discuss potential polyQ SCA therapeutic interventions in myelinating glial.
人类研究结合动物和细胞模型表明，神经胶质细胞是神经退行性疾病的主要贡献者，也是有前途的治疗靶点。在神经胶质细胞中，少突胶质细胞和施万细胞分别是中枢神经系统和周围神经系统的髓鞘形成神经胶质细胞。在这篇综述中，我们讨论了这些中枢和周围髓鞘形成神经胶质细胞对多聚谷氨酰胺（polyQ）脊髓小脑共济失调（SCA）1、2、3、6、7 和 17 型的发病机制的贡献。首先，我们强调了少突胶质细胞在健康状态下的功能，以及它们在 polyQ SCA 患者和疾病模型系统中是如何被破坏的。然后我们介绍了施万细胞在外周神经功能和修复中的作用，以及它们在外周神经病中的可能作用 polyQ SCA。最后，我们讨论了髓鞘形成神经胶质细胞中潜在的 polyQ SCA 治疗干预措施。
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