Glial but not neuronal development in the cochleo-vestibular ganglion requires Sox10
Glial but not neuronal development in the cochleo-vestibular ganglion requires Sox10
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DOI：
10.1111/j.1471-4159.2010.06897.x
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发表时间：
2010-09-01
影响因子：
4.7
通讯作者：
Malgrange, Brigitte
中科院分区：
文献类型：
--
作者：
Breuskin, Ingrid;Bodson, Morgan;Malgrange, Brigitte
耳蜗-前庭神经节含有神经嵴源性胶质细胞和来自神经源性耳基板的感觉神经元。关于调节这种结构紧密协调发展的分子机制知之甚少。在这里，我们报告说，Sox 10，一个高迁移率组DNA结合域转录因子，需要适当的发展神经嵴细胞衍生物，是专门表达在迁移后的神经嵴细胞在耳蜗前庭神经节。使用Sox 10缺陷小鼠，我们证明，这种转录因子是必不可少的生存，但不是一代，迁移后的内耳内的神经嵴细胞。在没有这些神经嵴衍生细胞的情况下，我们研究了耳囊衍生的听觉神经元的存活。令人惊讶的是，听觉神经元的分化，感觉靶神经支配和生存是保守的，尽管没有神经胶质细胞。此外，脑源性神经营养因子的表达增加的Sox 10缺陷小鼠的毛细胞，一种补偿机制，可以防止螺旋神经节神经元细胞死亡。两者合计，这些数据表明，在神经嵴源性胶质细胞的情况下，增加营养支持毛细胞促进Sox 10突变小鼠的螺旋神经节神经元的存活。
The cochleo-vestibular ganglion contains neural crest-derived glial cells and sensory neurons that are derived from the neurogenic otic placode. Little is known about the molecular mechanisms that regulate the tightly orchestrated development of this structure. Here, we report that Sox10, a high-mobility group DNA-binding domain transcription factor that is required for the proper development of neural crest cell derivatives, is specifically expressed in post-migratory neural crest cells in the cochleo-vestibular ganglion. Using Sox10-deficient mice, we demonstrate that this transcription factor is essential for the survival, but not the generation, of the post-migratory neural crest cells within the inner ear. In the absence of these neural crest-derived cells, we have investigated the survival of the otocyst-derived auditory neurons. Surprisingly, auditory neuron differentiation, sensory target innervation and survival are conserved despite the absence of glial cells. Moreover, brain-derived neurotrophic factor expression is increased in the hair cells of Sox10-deficient mice, a compensatory mechanism that may prevent spiral ganglion neuronal cell death. Taken together, these data suggest that in the absence of neural crest-derived glial cells, an increase trophic support from hair cells promotes the survival of spiral ganglion neurons in Sox10 mutant mice.