Institut für Biochemie, Emil-Fischer-Zentrum, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany.
Glia. 2012 May;60(5):806-19. doi: 10.1002/glia.22310. Epub 2012 Feb 15.
The transcription factor Sox10 is expressed throughout Schwann cell development and has already been shown to be essential for specification and for the identity and further development of immature Schwann cells. Here, we show that Sox10 is also required in Schwann cells for establishing the myelinating state. This is concluded from the fact that a peripheral neuropathy develops in mice in which Sox10 is deleted by a Cre recombinase whose expression is under control of Krox20 regulatory elements. This neuropathy is characterized by altered marker gene expression along the peripheral nerve, decreased conductivity, and severe persistent hypomyelination. As the Cre recombinase is additionally active in boundary cap cells, we also analyzed the role of Sox10 during embryogenesis in establishment and maintenance of the boundary between central and peripheral nervous systems. Sox10 deletion did not affect establishment or survival of boundary cap cells but appeared to compromise barrier function as cells expressing oligodendrocyte and astrocyte markers were no longer restricted to the central nervous system, and instead found in peripheral nerves. We infer that in addition to its many roles in Schwann cells, Sox10 is also important for the integrity of the boundary between central and peripheral nervous systems.
转录因子 Sox10 在施万细胞发育过程中表达，并已被证明对施万细胞的特化以及不成熟施万细胞的身份和进一步发育是必不可少的。在这里，我们表明 Sox10 对于建立髓鞘形成状态也是施万细胞所必需的。这是从 Sox10 被一种 Cre 重组酶删除的小鼠中出现周围神经病的事实中得出的结论，该 Cre 重组酶的表达受 Krox20 调节元件的控制。这种神经病的特征是沿周围神经改变标记基因的表达，电导率降低，严重持续的少突胶质细胞形成不足。由于 Cre 重组酶在边界帽细胞中也具有活性，我们还分析了 Sox10 在胚胎发生过程中在建立和维持中枢神经系统和周围神经系统之间边界中的作用。Sox10 缺失并不影响边界帽细胞的建立或存活，但似乎损害了屏障功能，因为表达少突胶质细胞和星形胶质细胞标记的细胞不再局限于中枢神经系统，而是在周围神经中发现。我们推断，Sox10 除了在施万细胞中具有许多作用外，对于中枢神经系统和周围神经系统之间边界的完整性也是重要的。
