Downregulation of otospiralin, a novel inner ear protein, causes hair cell degeneration and deafness

Journal of Neuroscience

Volumn 22, Issue 5, 2002, Pages 1718-1725

  Delprat, Benjamin ^a   Boulanger, Ana ^a,c   Wang, Jing ^a   Beaudoin, Vicky ^b   Guitton, Matthieu J ^a   Ventéo, Stéphanie ^a   Dechesne, Claude J ^a   Pujol, Rémy ^a   Lavigne Rebillard, Mireille ^a   Puel, Jean Luc ^a   Hamel, Christian P ^a  

^a INSERM   (France)

^b UNIVERSITÉ DU QUÉBEC À TROIS RIVIÈRES   (Canada)

^c NATIONAL EYE INSTITUTE   (United States)

Author keywords

Antisense; Cochlea; Deafness; Gene; Hair cell; Inner ear; Otospiralin; Vestibule

Indexed keywords

ANTISENSE OLIGONUCLEOTIDE; GAG PROTEIN; NUCLEOCAPSID PROTEIN; OTOSPIRALIN; PROTEIN; PROTEIN P30; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL TISSUE; ARTICLE; CELL SURVIVAL; COCHLEA; CONTROLLED STUDY; DOWN REGULATION; ELECTROPHYSIOLOGY; GUINEA PIG; HAIR CELL; HEARING IMPAIRMENT; IMMUNOHISTOCHEMISTRY; INNER EAR; ION TRANSPORT; MESENCHYME; NEUROEPITHELIUM; NONHUMAN; PRIORITY JOURNAL; PROTEIN MOTIF; RAT; SEMICIRCULAR CANAL; VESTIBULAR LABYRINTH; VESTIBULE;

EID: 0036523109     PISSN: 02706474     EISSN: None     Source Type: Journal    
DOI: 10.1523/jneurosci.22-05-01718.2002     Document Type: Article

References (48)

1. Alagramam KN, Zahorsky-Reeves J, Wright CG, Pawlowski KS, Erway LC, Stubbs L, Woychik RP (2000) Neuroepithelial defects of the inner ear in a new allele of the mouse mutation Ames waltzer. Hear Res 148:181-191.
2. Chomczynski P, Sacchi N (1987) Single-step method of RNA isolation by acid guanidinium thiocyanate-phenol-chloroform extraction. Anal Biochem 162:156-159.
3. Cohen-Salmon, M, Frenz, D, Liu, W, Verpy, E, Voegeling, S, Petit C (2000) Fdp, a new fibrocyte-derived protein related to M1A/CD-RAP, has an in vitro effect on the early differentiation of the inner ear mesenchyme. J Biol Chem 275:40036-40041.
4. Couloigner V, Fay M, Djelidi S, Farman N, Escoubet B, Runembert I, Sterkers O, Friedlander G, Ferrary E (2001) Location and function of the epithelial Na channel in the cochlea. Am J Physiol Renal Physiol 280:F214-222.
5. Delpire E, Lu J, England R, Dull C, Thorne T (1999) Deafness and imbalance associated with inactivation of the secretory Na-K-2Cl cotransporter. Nat Genet 22:192-195.
6. Everett LA, Morsli H, Wu DK, Green ED (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. Folz RJ, Nothwehr SF, Gordon JI (1988) Substrate specificity of eukaryotic signal peptidase. Site-saturation mutagenesis at position -1 regulates cleavage between multiple sites in human pre (delta pro) apolipoprotein A-II. J Biol Chem 263:2070-2078.
8. Fransen E, Verstreken M, Verhagen WI, Wuyts FL, Huygen PL, D'Haese P, Robertson NG, Morton CC, McGuirt WT, Smith RJ, Declau F, Van de Heyning PH, Van Camp G (1999) High prevalence of symptoms of Ménière's disease in three families with a mutation in the COCH gene. Hum Mol Genet 8:1425-1429.
9. Harter C, Ripoll C, Lenoir M, Hamel CP, Rebillard G (1999) Expression pattern of mammalian cochlea outer hair cell (OHC) mRNA: Screening of a rat OHC cDNA library. DNA Cell Biol 18:1-10.
10. Hawkins Jr JE (1959) The ototoxicity of kanamycin. Ann Otol Rhinol Laryngol 68:698-715.
11. Kikuchi T, Kimura RS, Paul DL, Takasaka T, Adams JC (2000) Gap junction systems in the mammalian cochlea. Brain Res Brain Res Rev 32:163-166.
12. Knipper M, Bandtlow C, Gestwa L, Kopschall I, Rohbock K, Wiechers B, Zenner HP, Zimmermann U (1998) Thyroid hormone affects Schwann cell and oligodendrocyte gene expression at the glial transition zone of the VIIIth nerve prior to cochlea function. Development 125:3709-3718.
13. Kyte J, Doolittle RF (1982) A simple method for displaying the hydropathic character of a protein. J Mol Biol 157:105-132.
14. Laemmli UK (1970) Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature 227:680-685.
15. Lautermann J, Ten Cate WJ, Altenhoff P, Grummer R, Traub O, Frank H, Jahnke K, Winterhager E (1998) Expression of the gap-junction connexins 26 and 30 in the rat cochlea. Cell Tissue Res 294:415-420.
16. Lenoir M, Puel J-L (1987) Dose-dependent changes in the rat cochlea following aminoglycoside intoxication. Histological study. Hear Res 26:199-209.
17. Lenoir M, Daudet N, Humbert G, Renard N, Gallego M, Pujol R, Eybalin M, Vago P (1999) Morphological and molecular changes in the inner hair cell region of the rat cochlea after amikacin treatment. J Neurocytol 28:925-937.
18. Littlewood Evans A, Müller U (2000) Stereocilia defects in the sensory hair cells of the inner ear in mice deficient in integrin α8β1. Nat Genet 27:424-428.
19. Miller AD, Verma IM (1984) Two base changes restore infectivity to a noninfectious molecular clone of moloney murine leukemia virus (pMLV-1). J Virol 49:214-222.
20. Mothe AJ, Brown IR (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.
21. Osako S, Hilding DA (1971) Electron microscopic studies of capillary permeability in normal and Ames waltzer deaf mice. Acta Otolaryngol 71:365-376.
22. Probst FJ, Fridell RA, Raphael Y, Saunders TL, Wang A, Liang Y, Morell RJ, Touchman JW, Lyons RH, Noben-Trauth K, Friedman TB, Camper SA (1998) Correction of deafness in shaker-2 mice by an unconventional myosin in a BAC transgene. Science 280:1444-1447.
23. Rendtorff ND, Frödin M, Attié-Bitach T, Vekemans M, Tommerup N (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.
24. Robertson NG, Lu L, Heller S, Merchant SN, Eavey RD, McKenna M, Nadol Jr JB, Miyamoto RT, Linthicum Jr FH, Lubianca Neto JF, Hudspeth AJ, Seidman CE, Morton CC, Seidman JG (1998) Mutations in a novel cochlear gene cause DFNA9, a human nonsyndromic deafness with vestibular dysfunction. Nat Genet 20:299-303.
25. Robertson NG, Heller S, Lin JS, Resendes BL, Weremowicz S, Denis CS, Bell AM, Hudspeth AJ, Morton CC (2000) A novel conserved cochlear gene. OTOR: Identification, expression analysis, and chromosomal mapping. Genomics 66:242-248.
26. Sage C, Ventéo S, Jeromin A, Roder J, Dechesne CJ (2000) Distribution of frequenin in the mouse inner ear during development, comparison with other calcium-binding proteins and synaptophysin. Hear Res 150:70-82.
27. Sakaguchi N, Crouch JJ, Lytle C, Schulte BA (1998) Na-K-Cl cotransporter expression in the developing and senescent gerbil cochlea. Hear Res 118:114-122.
28. Schägger H, von Jagow G (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.
29. Schwartzberg P, Colicelli J, Gordon ML, Goff SP (1984) Mutations in the gag gene of moloney murine leukemia virus: Effects on production of virions and reverse transcriptase. J Virol 49:918-924.
30. Scott DA, Wang R, Kreman TM, Sheffield VC, Karniski LP (1999) The Pendred syndrome gene encodes a chloride-iodide transport protein. Nat Genet 21:440-443.
31. Self T, Sobe T, Copeland NG, Jenkins NA, Avraham KB, Steel KP (1999) Role of myosin VI in the differentiation of cochlear hair cells. Dev Biol 214:331-341.
32. Shnerson A, Lenoir M, Van de Water TR, Pujol R (1983) The pattern of sensorineural degeneration in the cochlea of the deaf shaker-1 mouse: Ultrastructural observations. Dev Brain Res 9:305-315.
33. Sjöström B, Anniko M (1990) Morphologically specific vestibular hair cell degeneration in the jerker mutant mouse. Eur Arch Otorhinolaryngol 247:51-55.
34. Soto-Prior A, Lavigne-Rebillard M, Lenoir M, Ripoll C, Rebillard G, Vago P, Pujol R, Hamel CP (1997) Identification of preferentially expressed cochlear genes by systematic sequencing of a rat cochlea cDNA library. Mol Brain Res 47:1-10.
35. Spicer SS, Schulte BA (1991) Differentiation of inner ear fibrocytes according to their ion transport related activity. Hear Res 56:53-64.
36. Spicer SS, Schulte BA, Adams JC (1990) Immunolocalization of Na+,K(+)-ATPase and carbonic anhydrase in the gerbil's vestibular system. Hear Res 43:205-217.
37. Spicer SS, Gratton MA, Schulte BA (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.
38. Spoendlin H, Brun JP (1973) Relation of structural damage to response time and intensity in acoustic trauma. Acta Otolaryngol 75:220-226.
39. Stankovic KM, Adams JC, Brown D (1995) Immunolocalization of aquaporin CHIP in the guinea pig inner ear. Am J Physiol 269:C1450-1456.
40. 2+-ATPase gene cause deafness in deafwaddler mice. Nat Genet 19:390-394.
41. Thomadakis G, Ramoshebi LN, Crooks J, Rueger DC, Ripamonti U (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.
42. Towbin H, Staehelin T, Gordon J (1979) Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: Procedure and some applications. Proc Natl Acad Sci USA 76:4350-4354.
43. Tsuprun V, Santi P (1999) Ultrastructure and immunohistochemical identification of the extracellular matrix of the chinchilla cochlea. Hear Res 129:35-49.
44. Vetter DE, Mann JR, Wangemann P, Liu J, McLaughlin KJ, Lesage F, Marcus DC, Lazdunski M, Heinemann SF, Barhanin J (1996) Inner ear defects induced by null mutation of the isk gene. Neuron 17:1251-1264.
45. Weinberger DG, Ten Cate WJ, Lautermann J, Baethmann M (1999) Localization of laminin isoforms in the guinea pig cochlea. Laryngoscope 109:2001-2004.
46. Wilcox ER, Fex J (1992) Construction of a cDNA library from microdissected guinea pig organ of Corti. Hear Res 62:124-126.
47. Xia AP, Ikeda K, Katori Y, Oshima T, Kikuchi T, Takasaka T (2000) Expression of connexin 31 in the developing mouse cochlea. Neuro-Report 11:2449-2453.
48. Zheng L, Sekerkova G, Vranich K, Tilney LG, Mugnaini E, Bartles JR (2000) The deaf jerker mouse has a mutation in the gene encoding the espin actin-bundling proteins of hair cell stereocilia and lacks espins. Cell 102:3776-3785.