Deafness and cochlear fibrocyte alterations in mice deficient for the inner ear protein otospiralin

Molecular and Cellular Biology

Volumn 25, Issue 2, 2005, Pages 847-853

  Delprat, Benjamin ^a,b   Ruel, Jérôme ^a   Guitton, Matthieu J ^a   Hamard, Ghyslaine ^a,c   Lenoir, Marc ^a   Pujol, Rémy ^a   Puel, Jean Luc ^a   Brabet, Philippe ^a   Hamel, Christian P ^a  

^a HÔPITAL SAINT ELOI   (France)

^b Institut Pasteur   (France)

^c INSTITUT COCHIN   (France)

Author keywords

[No Author keywords available]

Indexed keywords

PROTEIN; PROTEIN OTOS; PROTEIN OTOSPIRALIN; UNCLASSIFIED DRUG;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL DEGENERATION; COCHLEA; COCHLEAR NERVE; CONTROLLED STUDY; GENE DELETION; HAIR CELL; HEARING IMPAIRMENT; HISTOPATHOLOGY; INNER EAR DISEASE; MOUSE; NERVE POTENTIAL; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEPLETION; PROTEIN EXPRESSION; STRIA VASCULARIS; VESTIBULE;

ANIMALS; AUDITORY THRESHOLD; COCHLEA; DEAFNESS; ELECTROPHYSIOLOGY; GENE TARGETING; HUMANS; MICE; MICE, KNOCKOUT; PROTEINS;

ANIMALIA; MAMMALIA;

EID: 11844301284     PISSN: 02707306     EISSN: None     Source Type: Journal    
DOI: 10.1128/MCB.25.2.847-853.2005     Document Type: Article

References (39)

1. 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.
2. 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.
3. 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.
4. 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.
5. 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.
6. 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.
7. 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.
8. Henson, M. M., and O. W. Henson. 1988. Tension fibroblasts and the connective tissue matrix of the spiral ligament. Hear. Res. 35:237-258.
9. 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.
10. 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.
11. Laemmli, U. K. 1970. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature 227:680-685.
12. 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.
13. 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.
14. 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.
15. 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.
16. 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.
17. 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.
18. 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.
19. 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.
20. 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.
21. 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.
22. 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.
23. 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.
24. 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.
25. 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.
26. 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.
27. 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.
28. Spicer, S. S., and B. A. Schulte. 1998. Evidence for a medial K+ recycling pathway from inner hair cells. Hear. Res. 118:1-12.
29. Spicer, S. S., and B. A. Schulte. 2002. Spiral ligament pathology in quiet-aged gerbils. Hear. Res. 172:172-185.
30. 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.
31. 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.
32. 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.
33. Tsuprun, V., and P. Santi. 1999. Ultrastructure and immunohistochemical identification of the extracellular matrix of the chinchilla cochlea. Hear. Res. 129:35-49.
34. Wangemann, P. 2002. K+ cycling and the endocochlear potential. Hear. Res. 165:1-9.
35. 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.
36. 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.
37. 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.
38. 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.
39. 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.