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

Mengqing Xiang, Lin Gan, Daqing Li, Zhi Yong Chen, Lijuan Zhou, Bert W. O'Malley, William Klein, Jeremy Nathans

Research output: Contribution to journalArticle

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

Original languageEnglish (US)
Pages (from-to)9445-9450
Number of pages6
JournalProceedings of the National Academy of Sciences of the United States of America
Volume94
Issue number17
DOIs
StatePublished - Aug 19 1997
Externally publishedYes

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Auditory Hair Cells
POU Domain Factors
Vestibular Hair Cells
Spiral Ganglion
Neurons
Genes
Trigeminal Ganglion
Retinal Ganglion Cells
Sensory Receptor Cells
Inner Ear
Brain Stem
Proteins
Phenotype

Keywords

  • Inner ear development

ASJC Scopus subject areas

  • General

Cite this

Essential role of POU-domain factor Brn-3c in auditory and vestibular hair cell development. / Xiang, Mengqing; Gan, Lin; Li, Daqing; Chen, Zhi Yong; Zhou, Lijuan; O'Malley, Bert W.; Klein, William; Nathans, Jeremy.

In: Proceedings of the National Academy of Sciences of the United States of America, Vol. 94, No. 17, 19.08.1997, p. 9445-9450.

Research output: Contribution to journalArticle

Xiang, Mengqing ; Gan, Lin ; Li, Daqing ; Chen, Zhi Yong ; Zhou, Lijuan ; O'Malley, Bert W. ; Klein, William ; Nathans, Jeremy. / Essential role of POU-domain factor Brn-3c in auditory and vestibular hair cell development. In: Proceedings of the National Academy of Sciences of the United States of America. 1997 ; Vol. 94, No. 17. pp. 9445-9450.
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