RT Journal Article
SR Electronic
T1 Downregulation of Otospiralin, a Novel Inner Ear Protein, Causes Hair Cell Degeneration and Deafness
JF The Journal of Neuroscience
JO J. Neurosci.
FD Society for Neuroscience
SP 1718
OP 1725
DO 10.1523/JNEUROSCI.22-05-01718.2002
VO 22
IS 5
A1 Benjamin Delprat
A1 Ana Boulanger
A1 Jing Wang
A1 Vicky Beaudoin
A1 Matthieu J. Guitton
A1 Stéphanie Ventéo
A1 Claude J. Dechesne
A1 Rémy Pujol
A1 Mireille Lavigne-Rebillard
A1 Jean-Luc Puel
A1 Christian P. Hamel
YR 2002
UL http://www.jneurosci.org/content/22/5/1718.abstract
AB 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.