Genetic insights into the morphogenesis of inner ear hair cells

Gregory I. Frolenkov, Inna A. Belyantseva, Thomas B. Friedman, Andrew J. Griffith

Research output: Contribution to journalReview articlepeer-review

173 Scopus citations


The mammalian inner ear is a sensory organ that has specialized hair cells that detect sound, as well as orientation and movement of the head. The 'hair' bundle on the apical surface of these cells is a mechanosensitive organelle that consists of precisely organized actin-filled projections known as stereocilia. Alterations in hair-bundle morphogenesis can result in hearing loss, balance defects or both. Positional cloning of genes that underlie hereditary hearing loss, coupled with the characterization of corresponding mouse models, is revealing how hair cells have adapted the molecular mechanisms of intracellular motility and intercellular adhesion for the morphogenesis of their apical surfaces.

Original languageEnglish
Pages (from-to)489-498
Number of pages10
JournalNature Reviews Genetics
Issue number7
StatePublished - Jul 2004

Bibliographical note

Funding Information:
We thank P. Belyantsev for the drawings and movies, R. Leapman for providing access to electron microscopy instruments, E. Boger for helpful discussions, and D. Drayna, R. Morell, M. Kelley and D. Wu for critically reading the manuscript. Work in the laboratories of T.B.F. and A.J.G. was supported by intramural research funds from the National Institute on Deafness and Other Communication Disorders.

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Genetics(clinical)


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