Ryan AF
Departments of Surgery/Otolaryngology and Neurosciences, UCSD School of Medicine and San Diego VA Medical Center, La Jolla, CA, 92093-0666, USA
Semin Cell Dev Biol. 1997 Jun;8(3):249-256. doi: 10.1006/scdb.1997.0146.
The development of the cells and tissues of the inner ear is controlled in part by the sequential expression of transcription factors, and recent studies have begun to define the roles played by such factors in morphogenesis of the labyrinth. A number of transcription factors have been shown to be expressed during inner ear development. Moreover, several genetic disorders of inner ear development in human and mouse have recently been identified as being caused by mutations of genes controlling transcription factors. In addition, the targeted mutation of several transcription factor genes influence the development of specific inner ear cell types, and suggest a critical role for these factors in controlling inner ear cell lineages. In particular, the POU-domain transcription factors are widely expressed in the developing ear. Gene deletion studies suggest that the Class IV POU-domain transcription factor Brn-3.1 is required for the development of auditory and vestibular hair cells, while Brn-3.0 is necessary for appropriate migration and numbers of spiral ganglion neurons.
内耳细胞和组织的发育部分受转录因子的顺序表达控制，最近的研究已开始明确这些因子在迷路形态发生中所起的作用。已证实多种转录因子在内耳发育过程中表达。此外，最近已确定人类和小鼠内耳发育的几种遗传疾病是由控制转录因子的基因突变引起的。另外，几种转录因子基因的靶向突变影响特定内耳细胞类型的发育，并表明这些因子在控制内耳细胞谱系中起关键作用。特别是，POU结构域转录因子在发育中的耳朵中广泛表达。基因缺失研究表明，IV类POU结构域转录因子Brn-3.1是听觉和前庭毛细胞发育所必需的，而Brn-3.0对于螺旋神经节神经元的适当迁移和数量是必需的。
