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首页> 外文期刊>The Journal of Neuroscience: The Official Journal of the Society for Neuroscience >Formation of compact myelin is required for maturation of the axonal cytoskeleton.
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Formation of compact myelin is required for maturation of the axonal cytoskeleton.

机译：轴突细胞骨架的成熟需要形成紧密髓鞘。

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摘要

Although traditional roles ascribed to myelinating glial cells are structural and supportive, the importance of compact myelin for proper functioning of the nervous system can be inferred from mutations in myelin proteins and neuropathologies associated with loss of myelin. Myelinating Schwann cells are known to affect local properties of peripheral axons (de Waegh et al., 1992), but little is known about effects of oligodendrocytes on CNS axons. The shiverer mutant mouse has a deletion in the myelin basic protein gene that eliminates compact myelin in the CNS. In shiverer mice, both local axonal features like phosphorylation of cytoskeletal proteins and neuronal perikaryon functions like cytoskeletal gene expression are altered. This leads to changes in the organization and composition of the axonal cytoskeleton in shiverer unmyelinated axons relative to age-matched wild-type myelinated fibers, although connectivity and patterns of neuronal activity are comparable. Remarkably, transgenic shiverer mice with thin myelin sheaths display an intermediate phenotype indicating that CNS neurons are sensitive to myelin sheath thickness. These results indicate that formation of a normal compact myelin sheath is required for normal maturation of the neuronal cytoskeleton in large CNS neurons.
机译：尽管归因于髓鞘神经胶质细胞的传统作用是结构性和支持性的，但紧密髓鞘蛋白对于神经系统正常功能的重要性可以从髓鞘蛋白的突变和与髓鞘蛋白丧失相关的神经病理学中推断出来。已知有髓鞘的雪旺氏细胞会影响周围轴突的局部特性（de Waegh等，1992），但对少突胶质细胞对中枢神经系统轴突的影响知之甚少。颤抖突变小鼠的髓鞘碱性蛋白基因缺失，从而消除了CNS中的紧密髓鞘。在颤抖的小鼠中，局部轴突特征（例如细胞骨架蛋白的磷酸化）和神经元周围核功能（例如细胞骨架基因表达）都被改变。相对于年龄匹配的野生型髓鞘纤维，这导致颤抖的无髓鞘轴突中轴突细胞骨架的组织和组成发生变化，尽管神经元活动的连通性和模式可比。值得注意的是，具有薄髓鞘的转基因颤抖小鼠表现出中间表型，表明中枢神经系统神经元对髓鞘厚度敏感。这些结果表明，大型CNS神经元中神经元细胞骨架的正常成熟需要正常致密髓鞘的形成。

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