EYA1-SIX1 complex in neurosensory cell fate induction in the mammalian inner ear

Hear Res. 2013 Mar;297:13-9. doi: 10.1016/j.heares.2012.09.009. Epub 2012 Oct 24.


The phosphatase-transactivator EYA1 interacts with the homeodomain protein SIX1 to form transcriptional activation complexes, which play essential roles in regulating cell proliferation, survival and induction of sensory and neuronal differentiation programs during inner ear development. Mutations of the Eya1 and Six1 genes cause profound developmental auditory defects in mice and humans. The molecular mechanisms and developmental processes controlled by the EYA1 and SIX1 complex in inner ear development and neurosensory fate induction are the focus of this review.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Animals
  • Cell Differentiation
  • Cell Lineage
  • Cell Proliferation
  • Cell Survival
  • Electroporation
  • Gene Expression Regulation, Developmental*
  • Hair Cells, Auditory / cytology
  • Homeodomain Proteins / physiology*
  • Humans
  • Intracellular Signaling Peptides and Proteins / physiology*
  • Mice
  • Mutation*
  • Neurons / metabolism*
  • Neurons / physiology
  • Nuclear Proteins / physiology*
  • Protein Tyrosine Phosphatases / physiology*
  • Proteomics / methods
  • Transcription, Genetic


  • Homeodomain Proteins
  • Intracellular Signaling Peptides and Proteins
  • Nuclear Proteins
  • Six1 protein, mouse
  • Eya1 protein, mouse
  • Protein Tyrosine Phosphatases