Molecular studies of hair cell development and survival

Audiol Neurootol. May-Jun 2002;7(3):138-40. doi: 10.1159/000058298.

Abstract

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.

MeSH terms

  • Animals
  • Cell Survival
  • Cloning, Organism
  • DNA-Binding Proteins / genetics*
  • Ear, Inner / growth & development
  • Ear, Inner / physiology
  • Gene Deletion
  • Hair Cells, Auditory / cytology
  • Hair Cells, Auditory / growth & development
  • Hair Cells, Auditory / physiology*
  • Hearing Disorders / genetics
  • Mice
  • Molecular Biology / methods*
  • Point Mutation / genetics
  • Transcription Factor Brn-3
  • Transcription Factor Brn-3A
  • Transcription Factors / genetics*

Substances

  • DNA-Binding Proteins
  • Pou4f1 protein, mouse
  • Transcription Factor Brn-3
  • Transcription Factor Brn-3A
  • Transcription Factors