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The Journal of Neuroscience, March 1, 2002, 22(5):1718-1725

Downregulation of Otospiralin, a Novel Inner Ear Protein, Causes Hair Cell Degeneration and Deafness

Benjamin Delprat^{1, *}, Ana Boulanger^{1, *}, Jing Wang¹, Vicky Beaudoin², Matthieu J. Guitton¹, Stéphanie Ventéo³, Claude J. Dechesne³, Rémy Pujol¹, Mireille Lavigne-Rebillard¹, Jean-Luc Puel¹, and Christian P. Hamel¹

¹ Institut National de la Santé et de la Recherche Médicale U. 254, Laboratoire de Neurobiologie de l'Audition, 34090 Montpellier, France, ² Département de Chimie-Biologie, Université du Québec à Trois-Rivières, Trois-Rivières (Québec), Canada, G9A 5H7, and ³ Institut National de la Santé et de la Recherche Médicale U. 432, Neurobiologie et Développement du Système Audiovestibulaire, Université Montpellier II, 34095 Montpellier cedex 05, France

Mesenchymal nonsensory regions of the inner ear are important structures surrounding the neurosensory epithelium that are believed to participate in the ionic homeostasis of the cochlea and vestibule. We report here the discovery of otospiralin, an inner ear-specific protein that is produced by fibrocytes from these regions, including the spiral ligament and spiral limbus in the cochlea and the maculae and semicircular canals in the vestibule. Otospiralin is a novel 6.4 kDa protein of unknown function that shares a protein motif with the gag p30 core shell nucleocapsid protein of type C retroviruses. To evaluate its functional importance, we downregulated otospiralin by cochlear perfusion of antisense oligonucleotides in guinea pigs. This led to a rapid threshold elevation of the compound action potentials and irreversible deafness. Cochlear examination by transmission electron microscopy revealed hair cell loss and degeneration of the organ of Corti. This demonstrates that otospiralin is essential for the survival of the neurosensory epithelium.

Key words: otospiralin; cochlea; vestibule; hair cell; deafness; gene; antisense; inner ear

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^* B.D. and A.B. contributed equally to this work

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Copyright © 2002 Society for Neuroscience  0270-6474/02/2251718-08$05.00/0

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				M.-O. Trowe, H. Maier, M. Schweizer, and A. Kispert Deafness in mice lacking the T-box transcription factor Tbx18 in otic fibrocytes Development, May 1, 2008; 135(9): 1725 - 1734. [Abstract] [Full Text] [PDF]

				B. Delprat, J. Ruel, M. J. Guitton, G. Hamard, M. Lenoir, R. Pujol, J.-L. Puel, P. Brabet, and C. P. Hamel Deafness and Cochlear Fibrocyte Alterations in Mice Deficient for the Inner Ear Protein Otospiralin Mol. Cell. Biol., January 15, 2005; 25(2): 847 - 853. [Abstract] [Full Text] [PDF]

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