Physical and functional interaction between protocadherin 15 and myosin VIIa in mechanosensory hair cells

Mathias Senften, Martin Schwander, Piotr Kazmierczak, Concepcion Lillo, Jung Bum Shin, Tama Hasson, Gwenaëlle S.G. Géléoc, Peter G. Gillespie, David Williams, Jeffrey R. Holt, Ulrich Müller

Research output: Contribution to journalArticle

114 Scopus citations

Abstract

Hair cells of the mammalian inner ear are the mechanoreceptors that convert sound-induced vibrations into electrical signals. The molecular mechanisms that regulate the development and function of the mechanically sensitive organelle of hair cells, the hair bundle, are poorly defined. We link here two gene products that have been associated with deafness and hair bundle defects, protocadherin 15 (PCDH15) and myosin VIIa (MYO7A), into a common pathway. We show that PCDH15 binds to MYO7A and that both proteins are expressed in an overlapping pattern in hair bundles. PCDH15 localization is perturbed in MYO7A-deficient mice, whereas MYO7A localization is perturbed in PCDH15-deficient mice. Like MYO7A, PCDH15 is critical for the development of hair bundles in cochlear and vestibular hair cells, controlling hair bundle morphogenesis and polarity. Cochlear and vestibular hair cells from PCDH15-deficient mice also show defects in mechanotransduction. Together, our findings suggest that PCDH15 and MYO7A cooperate to regulate the development and function of the mechanically sensitive hair bundle.

Original languageEnglish (US)
Pages (from-to)2060-2071
Number of pages12
JournalJournal of Neuroscience
Volume26
Issue number7
DOIs
StatePublished - Feb 15 2006

Keywords

  • Cochlea
  • Deafness
  • Hair cell
  • MYO7A
  • Mechanotransduction
  • PCDH15

ASJC Scopus subject areas

  • Neuroscience(all)

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    Senften, M., Schwander, M., Kazmierczak, P., Lillo, C., Shin, J. B., Hasson, T., Géléoc, G. S. G., Gillespie, P. G., Williams, D., Holt, J. R., & Müller, U. (2006). Physical and functional interaction between protocadherin 15 and myosin VIIa in mechanosensory hair cells. Journal of Neuroscience, 26(7), 2060-2071. https://doi.org/10.1523/JNEUROSCI.4251-05.2006