Myosin VIIa, harmonin and cadherin23, three Usher I gene products cooperate to shape the sensory hair bundle

Boëda, Batiste, El-Amraoui, Aziz, Bahloul, Amel, Goodyear, Richard, Daviet, Laurent, Blanchard, Stéphane, Perfettini, Isabelle, Fath, Karl R, Shorte, Spencer, Reiners, Jan, Houdusse, Anne, Legrain, Pierre, Wolfrum, Uwe, Richardson, Guy and Petit, Christine (2002) Myosin VIIa, harmonin and cadherin23, three Usher I gene products cooperate to shape the sensory hair bundle. EMBO Journal, 21 (24). 6689 - 6699. ISSN 0261-4189

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Abstract

Deaf-blindness in three distinct genetic forms of Usher type I syndrome (USH1) is caused by defects in myosin VIIa, harmonin and cadherin 23. Despite being critical for hearing, the functions of these proteins in the inner ear remain elusive. Here we show that harmonin, a PDZ domain-containing protein, and cadherin 23 are both present in the growing stereocilia and that they bind to each other. Moreover, we demonstrate that harmonin b is an F-actin-bundling protein, which is thus likely to anchor cadherin 23 to the stereocilia microfilaments, thereby identifying a novel anchorage mode of the cadherins to the actin cytoskeleton. Moreover, harmonin b interacts directly with myosin VIIa, and is absent from the disorganized hair bundles of myosin VIIa mutant mice, suggesting that myosin VIIa conveys harmonin b along the actin core of the developing stereocilia. We propose that the shaping of the hair bundle relies on a functional unit composed of myosin VIIa, harmonin b and cadherin 23 that is essential to ensure the cohesion of the stereocilia.

Item Type: Article
Schools and Departments: School of Life Sciences > Neuroscience
Depositing User: Richard Goodyear
Date Deposited: 06 Feb 2012 20:23
Last Modified: 26 Jun 2012 14:47
URI: http://sro.sussex.ac.uk/id/eprint/25686
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