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

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.

Pubmed ID: 9256502

Authors

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

Journal

Proceedings of the National Academy of Sciences of the United States of America

Publication Data

August 19, 1997

Associated Grants

None

Mesh Terms

  • 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