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

Journal:

Proc. Natl. Acad. Sci. U.S.A. 1997 Aug

Authors:

Xiang M, Gan L, Li D, Chen ZY, Zhou L, O'Malley BW, Klein W, Nathans J

Abstract

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 c
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arrying 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-3c phenotype reported here demonstrates that each of the Brn-3 genes plays distinctive roles in the somatosensory, visual, and auditory/vestibular systems.[less]

Mesh Headings:

Animals, DNA-Binding Proteins, Ear, Inner, Female, Gene Expression Regulation, Developmental, Hair Cells, Vestibular, Mice, Mice, Mutant Strains, Pregnancy, Transcription Factor Brn-3, Transcription Factor Brn-3A, Transcription Factor Brn-3B, Transcription Factor Brn-3C, Transcription Factors