Transcription factors that control inner ear development and their potential for transdifferentiation and reprogramming

Hear Res. 2013 Mar;297:84-90. doi: 10.1016/j.heares.2012.11.001. Epub 2012 Nov 14.


Transcription factors (TFs) participate during various processes throughout inner ear development such as induction, morphogenesis and determination of cell fate and differentiation. The analysis of mouse mutants has been essential to define the requirement of different members of TF families during these processes. Next to their roles during normal development TFs have also been tested for their capacity to induce differentiation or reprogram cells upon misexpression. Recently the capacity of TFs to transdifferentiate easily accessible cells such as fibroblasts to highly specialized cell types has opened a new pathway for regenerative therapies. In this review the influence of TFs acting during different phases and processes of inner ear development will be summarized. A special focus will be given to TFs with a potential to reprogram or transdifferentiate cells to sensory cell types of the inner ear such as hair cells or neurons and thus may form part of future protocols directed to generate replacement cells in a clinical context.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Basic Helix-Loop-Helix Transcription Factors / metabolism
  • Cell Differentiation
  • Ear, Inner / embryology*
  • Ear, Inner / growth & development
  • Ear, Inner / physiology*
  • Fibroblasts / cytology
  • Gene Expression Regulation, Developmental
  • Hair Cells, Auditory / physiology*
  • Humans
  • Intracellular Signaling Peptides and Proteins / metabolism
  • Mice
  • Mutation
  • Nerve Regeneration / physiology*
  • Neurons / metabolism
  • Nuclear Proteins / metabolism
  • Phenotype
  • Protein Tyrosine Phosphatases / metabolism
  • Transcription Factors / metabolism*


  • Atoh1 protein, mouse
  • Basic Helix-Loop-Helix Transcription Factors
  • Intracellular Signaling Peptides and Proteins
  • Nuclear Proteins
  • Transcription Factors
  • Eya1 protein, mouse
  • Protein Tyrosine Phosphatases