Essential role of POU-domain factor Brn-3c in auditory and vestibular hair cell development

被引:266
作者
Xiang, MQ
Gan, L
Li, DQ
Chen, ZY
Zhou, LJ
OMalley, BW
Klein, W
Nathans, J
机构
[1] JOHNS HOPKINS UNIV,SCH MED,DEPT GENET & MOL BIOL,BALTIMORE,MD 21205
[2] JOHNS HOPKINS UNIV,SCH MED,DEPT OTOLARYNGOL HEAD & NECK SURG,BALTIMORE,MD 21205
[3] JOHNS HOPKINS UNIV,SCH MED,DEPT NEUROSCI & OPHTHALMOL,BALTIMORE,MD 21205
[4] JOHNS HOPKINS UNIV,SCH MED,HOWARD HUGHES MED INST,BALTIMORE,MD 21205
[5] UNIV MED & DENT NEW JERSEY,ROBERT WOOD JOHNSON MED SCH,DEPT PEDIAT,CTR ADV BIOTECHNOL & MED,PISCATAWAY,NJ 08854
[6] UNIV TEXAS,MD ANDERSON CANCER CTR,DEPT BIOCHEM & MOL BIOL,HOUSTON,TX 77030
关键词
inner ear development;
D O I
10.1073/pnas.94.17.9445
中图分类号
O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];
学科分类号
07 ; 0710 ; 09 ;
摘要
The Brn-3 subfamily of POU-domain transcription factor genes consists of three highly homologous members-Brn-3a, Brn-3b, and Brn-3c-that are expressed in sensory neurons and in a small number of brainstem nuclei, This paper describes the role of Brn-3c in auditory and vestibular system development, In the inner ear, the Brn-3c protein is found only in auditory and vestibular hair cells, and the Brn-3a and Brn-3b proteins are found only in subsets of spiral and vestibular ganglion neurons, Mice carrying a targeted deletion of the Brn-3c gene are deaf and have impaired balance, These defects reflect a complete loss of auditory and vestibular hair cells during the late embryonic and early postnatal period and a secondary loss of spiral and vestibular ganglion neurons. Together with earlier work demonstrating a loss of trigeminal ganglion neurons and retinal ganglion cells in mice carrying targeted disruptions in the Brn-3a and Brn-3b genes, respectively, the Brn-Sc phenotype reported here demonstrates that each of the Brn-3 genes plays distinctive roles in the somatosensory, visual, and auditory/vestibular systems.
引用
收藏
页码:9445 / 9450
页数:6
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