Molecular studies of hair cell development and survival.
Molecular studies of hair cell development and survival.
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毛细胞发育和存活的分子研究。
DOI：
10.1159/000058298
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发表时间：
2002
影响因子：
1.6
通讯作者：
Ryan,AllenF
中科院分区：
文献类型：
--
作者：
Ryan,AllenF
听觉和前庭感觉上皮中毛细胞的发育是一个复杂的过程，涉及许多调节蛋白的协调表达。其中包括 POU 结构域转录因子 Brn-3.1。该因子在决定毛细胞命运后立即在毛细胞前体中表达，并持续终生。在小鼠中删除该因子会导致发育过程中毛细胞分化失败，并导致大多数未分化细胞死亡。 Brn-3.1 的正常表达对于成人毛细胞的生存也是必需的，因为该基因的突变会导致人类显性、晚发性、遗传性听力损失。 Brn-3.1 表达开始的时间表明调节其表达的因子可能参与毛细胞的命运决定。此外，本身受 Brn-3.1 直接调控的基因似乎在毛细胞发育和存活中发挥着关键作用。
The development of hair cells in both the auditory and vestibular sensory epithelia is a complex process that involves the coordinated expression of many regulatory proteins. Among these is the POU-domain transcription factor Brn-3.1. This factor is expressed in hair cell precursors immediately after commitment to the hair cell fate, and continues throughout life. Deletion of this factor in mice leads to failure of hair cell differentiation during development, and to the death of a majority of the undifferentiated cells. Normal expression of Brn-3.1 is required for adult hair cell survival as well, since a mutation in this gene causes dominant, late-onset, inherited hearing loss in humans. The timing of the onset of Brn-3.1 expression suggests that factors regulating its expression may be involved in fate determination of hair cells. Moreover, genes that are themselves directly regulated by Brn-3.1 appear to play critical roles in hair cell development and survival.