This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrowReprints and Permissions
Right arrow Copyright Information
Right arrow Books from ASM Press
Right arrow MicrobeWorld
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Delprat, B.
Right arrow Articles by Hamel, C. P.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Delprat, B.
Right arrow Articles by Hamel, C. P.

 Previous Article  |  Next Article 

Molecular and Cellular Biology, January 2005, p. 847-853, Vol. 25, No. 2
0270-7306/05/$08.00+0     doi:10.1128/MCB.25.2.847-853.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.

Deafness and Cochlear Fibrocyte Alterations in Mice Deficient for the Inner Ear Protein Otospiralin

Benjamin Delprat,* Jérôme Ruel, Matthieu J. Guitton, Ghyslaine Hamard,{dagger} Marc Lenoir, Rémy Pujol, Jean-Luc Puel, Philippe Brabet, and Christian P. Hamel*

INSERM, U.583, Physiopathologie et Thérapie des Déficits Sensoriels et Moteurs, Institut des Neurosciences de Montpellier, Hôpital Saint-Eloi, Montpellier, France

Received 26 June 2004/ Returned for modification 16 September 2004/ Accepted 10 October 2004

In the cochlea, the mammalian auditory organ, fibrocytes of the mesenchymal nonsensory regions play important roles in cochlear physiology, including the maintenance of ionic and hydric components in the endolymph. Occurrence of human deafness in fibrocyte alterations underlines their critical roles in auditory function. We recently described a novel gene, Otos, which encodes otospiralin, a small protein of unknown function that is produced by the fibrocytes of the cochlea and vestibule. We now have generated mice with deletion of Otos and found that they show moderate deafness, with no frequency predominance. Histopathology revealed a degeneration of type II and IV fibrocytes, while hair cells and stria vascularis appeared normal. Together, these findings suggest that impairment of fibrocytes caused by the loss in otospiralin leads to abnormal cochlear physiology and auditory function. This moderate dysfunction may predispose to age-related hearing loss.

* Corresponding author. Mailing address for Christian P. Hamel: INSERM U.583, Physiopathologie et Thérapie des Déficits Sensoriels et Moteurs, Institut des Neurosciences de Montpellier, Hôpital Saint-Eloi, BP 74103, 80, rue Augustin Fliche, 34295 Montpellier cedex 05, France. Phone: 33 499 636 010. Fax: 33 499 636 020. E-mail: hamel{at}montp.inserm.fr. Mailing address for Benjamin Delprat: INSERM U. 587, Génétique des Déficits Sensoriels, 25 rue du Dr Roux, 75724 Paris cedex 15, France. E-mail: delprat{at}pasteur.fr.

{dagger} Present address: INSERM U.567, Institut Cochin, 75014 Paris, France.

Molecular and Cellular Biology, January 2005, p. 847-853, Vol. 25, No. 2
0022-538X/05/$08.00+0     doi:10.1128/MCB.25.2.847-853.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.



This article has been cited by other articles:

  • Lopez, I. A., Rosenblatt, M. I., Kim, C., Galbraith, G. C., Jones, S. M., Kao, L., Newman, D., Liu, W., Yeh, S., Pushkin, A., Abuladze, N., Kurtz, I. (2009). Slc4a11 Gene Disruption in Mice: CELLULAR TARGETS OF SENSORINEURONAL ABNORMALITIES. J. Biol. Chem. 284: 26882-26896 [Abstract] [Full Text]  
  • Trowe, M.-O., Maier, H., Schweizer, M., Kispert, A. (2008). Deafness in mice lacking the T-box transcription factor Tbx18 in otic fibrocytes. Development 135: 1725-1734 [Abstract] [Full Text]  
  • Kamiya, K., Fujinami, Y., Hoya, N., Okamoto, Y., Kouike, H., Komatsuzaki, R., Kusano, R., Nakagawa, S., Satoh, H., Fujii, M., Matsunaga, T. (2007). Mesenchymal Stem Cell Transplantation Accelerates Hearing Recovery through the Repair of Injured Cochlear Fibrocytes. Am. J. Pathol. 171: 214-226 [Abstract] [Full Text]  
  • Gao, J., Maison, S. F., Wu, X., Hirose, K., Jones, S. M., Bayazitov, I., Tian, Y., Mittleman, G., Matthews, D. B., Zakharenko, S. S., Liberman, M. C., Zuo, J. (2007). Orphan Glutamate Receptor {delta}1 Subunit Required for High-Frequency Hearing. Mol. Cell. Biol. 27: 4500-4512 [Abstract] [Full Text]  


Copyright © 2009 by the American Society for Microbiology.   For an alternate route to Journals.ASM.org, visit: http://intl-journals.asm.org | More Info»