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

REFERENCES

• Cody, A. R., and I. J. Russell. 1987. The response of hair cells in the basal turn of the guinea-pig cochlea to tones. J. Physiol. 383:551–569.
   PubMed Web of Science ®Google Scholar
• Cohen-Salmon, M., D. Frenz, W. Liu, E. Verpy, S. Voegeling, and C. Petit. 2000. Fdp, a new fibrocyte-derived protein related to MIA/CD-RAP, has an in vitro effect on the early differentiation of the inner ear mesenchyme. J. Biol. Chem. 275:40036–40041.
   PubMedGoogle Scholar
• Couloigner, V., M. Fay, S. Djelidi, N. Farman, B. Escoubet, I. Runembert, O. Sterkers, G. Friedlander, and E. Ferrary. 2001. Location and function of the epithelial Na channel in the cochlea. Am. J. Physiol. Renal Physiol. 280:F214–F222.
   PubMedGoogle Scholar
• Delprat, B., A. Boulanger, J. Wang, V. Beaudoin, M. J. Guitton, S. Venteo, C. J. Dechesne, R. Pujol, M. Lavigne-Rebillard, J.-L. Puel, and C. P. Hamel. 2002. Downregulation of otospiralin, a novel inner ear protein, causes hair cell degeneration and deafness. J. Neurosci. 22:1718–1725.
   PubMed Web of Science ®Google Scholar
• Everett, L. A., B. Glaser, J. C. Beck, J. R. Idol, A. Buchs, M. Heyman, F. Adawi, E. Hazani, E. Nassir, A. D. Baxevanis, V. C. Sheffield, and E. D. Green. 1997. Pendred syndrome is caused by mutations in a putative sulphate transporter gene (PDS). Nat. Genet. 17:411–422.
   PubMed Web of Science ®Google Scholar
• Everett, L. A., H. Morsli, D. K. Wu, and E. D. Green. 1999. Expression pattern of the mouse ortholog of the Pendred's syndrome gene (Pds) suggests a key role for pendrin in the inner ear. Proc. Natl. Acad. Sci. USA 96:9727–9732.
   PubMed Web of Science ®Google Scholar
• Forge, A., D. Becker, S. Casalotti, J. Edwards, W. H. Evans, N. Lench, and M. Souter. 1999. Gap junctions and connexin expression in the inner ear. Novartis Found. Symp. 219:134–156.
   PubMed Web of Science ®Google Scholar
• Henson, M. M., and O. W. Henson. 1988. Tension fibroblasts and the connective tissue matrix of the spiral ligament. Hear. Res. 35:237–258.
   PubMed Web of Science ®Google Scholar
• Hequembourg, S., and M. C. Liberman. 2001. Spiral ligament pathology: a major aspect of age-related cochlear degeneration in C57BL/6 mice. J. Assoc. Res. Otolaryngol. 2:118–129.
   PubMed Web of Science ®Google Scholar
• Kikuchi, T., R. S. Kimura, D. L. Paul, T. Takasaka, and J. C. Adams. 2000. Gap junction systems in the mammalian cochlea. Brain Res. Brain Res. Rev. 32:163–166.
   PubMed Web of Science ®Google Scholar
• Laemmli, U. K. 1970. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature 227:680–685.
   PubMed Web of Science ®Google Scholar
• Lautermann, J., W. J. Ten Cate, P. Altenhoff, R. Grummer, O. Traub, H. Frank, K. Jahnke, and E. Winterhager. 1998. Expression of the gap-junction connexins 26 and 30 in the rat cochlea. Cell Tissue Res. 294:415–420.
   PubMed Web of Science ®Google Scholar
• Lavigne-Rebillard, M., B. Delprat, M.-O. Surget, J.-M. Griffoin, D. Weil, M. Arbones, R. Vincent, and C. P. Hamel. 2003. Gene structure, chromosomal localization, and mutation screening of the human gene for the inner ear protein otospiralin. Neurogenetics 4:137–140.
   PubMedGoogle Scholar
• Minowa, O., K. Ikeda, Y. Sugitani, T. Oshima, S. Nakai, Y. Katori, M. Suzuki, M. Furukawa, T. Kawase, Y. Zheng, M. Ogura, Y. Asada, K. Watanabe, H. Yamanaka, S. Gotoh, M. Nishi-Takeshima, T. Sugimoto, T. Kikuchi, T. Takasaka, and T. Noda. 1999. Altered cochlear fibrocytes in a mouse model of DFN3 nonsyndromic deafness. Science 285:1408–1411.
   PubMedGoogle Scholar
• Mothe, A. J., and I. R. Brown. 2001. Expression of mRNA encoding extracellular matrix glycoproteins SPARC and SC1 is temporally and spatially regulated in the developing cochlea of the rat inner ear. Hear. Res. 155:161–174.
   PubMedGoogle Scholar
• Pace, A. J., V. J. Madden, O. W. Henson, B. H. Koller, and M. M. Henson. 2001. Ultrastructure of the inner ear of NKCC1-deficient mice. Hear. Res. 156:17–30.
   PubMedGoogle Scholar
• Patuzzi, R. B., G. K. Yates, and B. M. Johnstone. 1989. The origin of the low-frequency microphonic in the first cochlear turn of guinea-pig. Hear. Res. 39:177–188.
   PubMed Web of Science ®Google Scholar
• Rendtorff, N. D., M. Frödin, T. Attié-Bitach, M. Vekemans, and N. Tommerup. 2001. Identification and characterization of an inner ear-expressed human melanoma inhibitory activity (MIA)-like gene (MIAL) with a frequent polymorphism that abolishes translation. Genomics 71:40–52.
   PubMedGoogle Scholar
• Robertson, N. G., L. Lu, S. Heller, S. N. Merchant, R. D. Eavey, M. McKenna, J. B. Nadol, R. T. Miyamoto, F. H. Linthicum, J. F. Lubianca Neto, A. J. Hudspeth, C. E. Seidman, C. C. Morton, and J. G. Seidman. 1998. Mutations in a novel cochlear gene cause DFNA9, a human nonsyndromic deafness with vestibular dysfunction. Nat. Genet. 20:299–303.
   PubMed Web of Science ®Google Scholar
• Robertson, N. G., S. Heller, J. S. Lin, B. L. Resendes, S. Weremowicz, C. S. Denis, A. M. Bell, A. J. Hudspeth, and C. C. Morton. 2000. A novel conserved cochlear gene, OTOR: identification, expression analysis, and chromosomal mapping. Genomics 66:242–248.
   PubMedGoogle Scholar
• Sakaguchi, N., J. J. Crouch, C. Lytle, and B. A. Schulte. 1998. Na-K-Cl cotransporter expression in the developing and senescent gerbil cochlea. Hear. Res. 118:114–122.
   PubMed Web of Science ®Google Scholar
• Schagger, H., and G. von Jagow. 1987. Tricine-sodium dodecyl sulfate-polyacrylamide gel electrophoresis for the separation of proteins in the range from 1 to 100 kDa. Anal. Biochem. 166:368–379.
   PubMed Web of Science ®Google Scholar
• Scott, D. A., R. Wang, T. M. Kreman, V. C. Sheffield, and L. P. Karniski. 1999. The Pendred syndrome gene encodes a chloride-iodide transport protein. Nat. Genet. 21:440–443.
   PubMed Web of Science ®Google Scholar
• Soto-Prior, A., M. Lavigne-Rebillard, M. Lenoir, C. Ripoll, G. Rebillard, P. Vago, R. Pujol, and C. P. Hamel. 1997. Identification of preferentially expressed cochlear genes by systematic sequencing of a rat cochlea cDNA library. Mol. Brain Res. 47:1–10.
   PubMedGoogle Scholar
• Spicer, S. S., B. A. Schulte, and J. C. Adams. 1990. Immunolocalization of Na+,K(+)-ATPase and carbonic anhydrase in the gerbil's vestibular system. Hear. Res. 43:205–217.
   PubMed Web of Science ®Google Scholar
• Spicer, S. S., and B. A. Schulte. 1991. Differentiation of inner ear fibrocytes according to their ion transport related activity. Hear. Res. 56:53–64.
   PubMed Web of Science ®Google Scholar
• Spicer, S. S., M. A. Gratton, and B. A. Schulte. 1997. Expression patterns of ion transport enzymes in spiral ligament fibrocytes change in relation to strial atrophy in the aged gerbil cochlea. Hear. Res. 111:93–102.
   PubMed Web of Science ®Google Scholar
• Spicer, S. S., and B. A. Schulte. 1998. Evidence for a medial K+ recycling pathway from inner hair cells. Hear. Res. 118:1–12.
   PubMed Web of Science ®Google Scholar
• Spicer, S. S., and B. A. Schulte. 2002. Spiral ligament pathology in quiet-aged gerbils. Hear. Res. 172:172–185.
   PubMedGoogle Scholar
• Stankovic, K. M., J. C. Adams, and D. Brown. 1995. Immunolocalization of aquaporin CHIP in the guinea pig inner ear. Am. J. Physiol. 269:C1450–C1456.
   PubMedGoogle Scholar
• Thomadakis, G., L. N. Ramoshebi, J. Crooks, D. C. Rueger, and U. Ripamonti. 1999. Immunolocalization of Bone Morphogenetic Protein-2 and -3 and Osteogenic Protein-1 during murine tooth root morphogenesis and in other craniofacial structures. Eur. J. Oral Sci. 107:368–377.
   PubMed Web of Science ®Google Scholar
• Towbin, H., T. Staehelin, and J. Gordon. 1979. Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications. Proc. Natl. Acad. Sci. USA 76:4350–4354.
   PubMed Web of Science ®Google Scholar
• Tsuprun, V., and P. Santi. 1999. Ultrastructure and immunohistochemical identification of the extracellular matrix of the chinchilla cochlea. Hear. Res. 129:35–49.
   PubMedGoogle Scholar
• Wangemann, P. 2002. K+ cycling and the endocochlear potential. Hear. Res. 165:1–9.
   PubMed Web of Science ®Google Scholar
• Weinberger, D. G., W. J. Ten Cate, J. Lautermann, and M. Baethmann. 1999. Localization of laminin isoforms in the guinea pig cochlea. Laryngoscope 109:2001–2004.
   PubMedGoogle Scholar
• Wu, T., and D. C. Marcus. 2003. Age-related changes in cochlear endolymphatic potassium and potential in CD-1 and CBA/CaJ mice. J. Assoc. Res. Otolaryngol. 4:353–362.
   PubMedGoogle Scholar
• Xia, A. P., K. Ikeda, Y. Katori, T. Oshima, T. Kikuchi, and T. Takasaka. 2000. Expression of connexin 31 in the developing mouse cochlea. Neuroreport 11:2449–2453.
   PubMed Web of Science ®Google Scholar
• Xia, A. P., T. Kikuchi, O. Minowa, Y. Katori, T. Oshima, T. Noda, and K. Ikeda. 2002. Late-onset hearing loss in a mouse model of DFN3 non-syndromic deafness: morphologic and immunohistochemical analyses. Hear. Res. 166:150–158.
   PubMedGoogle Scholar
• Zheng, Q. Y., K. R. Johnson, and L. C. Erway. 1999. Assessment of hearing in 80 inbred strains of mice by ABR threshold analyses. Hear. Res. 130:94–107.
   PubMed Web of Science ®Google Scholar