Cochlin expression in the rat perilymph during postnatal development

Acta Oto-Laryngologica

Volumn 132, Issue 11, 2012, Pages 1134-1139

  Shiiba, Kyoko ^a   Shindo, Susumu ^b   Ikezono, Tetsuo ^b   Sekine, Kuwon ^a   Matsumura, Tomohiro ^a   Sekiguchi, Satomi ^c   Yagi, Toshiaki ^d   Okubo, Kimihiro ^a  

^a NIPPON MEDICAL SCHOOL   (Japan)

^b SAITAMA MEDICAL UNIVERSITY   (Japan)

^c LSI MEDIENCE CORPORATION   (Japan)

^d UNIVERSITY OF HUMAN ENVIRONMENTS   (Japan)

Author keywords

COCH gene; DFNA9; Real time PCR; Western blot

Indexed keywords

COCHLIN; COCHLIN TOMOPROTEIN; ISOPROTEIN; MESSENGER RNA; PROTEIN P63; UNCLASSIFIED DRUG;

ANIMAL TISSUE; ARTICLE; INNER EAR; NONHUMAN; PERILYMPH; POSTNATAL DEVELOPMENT; PRIORITY JOURNAL; PROTEIN EXPRESSION; RAT; REAL TIME POLYMERASE CHAIN REACTION; WESTERN BLOTTING;

ANIMALS; BLOTTING, WESTERN; EAR; EXTRACELLULAR MATRIX PROTEINS; PERILYMPH; PROTEIN ISOFORMS; RATS; RATS, WISTAR; REAL-TIME POLYMERASE CHAIN REACTION;

EID: 84867492798     PISSN: 00016489     EISSN: 16512553     Source Type: Journal    
DOI: 10.3109/00016489.2012.687456     Document Type: Article

References (20)

1. Robertson NG, Lu L, Heller S, Merchant SN, Eavey RD, McKenna M, et al. Mutations in a novel cochlear gene cause DFNA9, a human nonsyndromic sensorineural deafness with vestibular dysfunction. Nat Genet 1998;20:299-303.
2. de Kok YJ, Bom SJ, Brunt TM, Kemperman MH, van Beusekom E, van der Velde-Visser SD, et al. A Pro51Ser mutation in the COCH gene is associated with late onset autosomal dominant progressive sensorineural hearing loss with vestibular defects.Hum MolGenet 1999;8:361-6.
3. Usami S, Takahashi K, Yuge I, Ohtsuka A, Namba A, Abe S, et al. Mutations in the COCH gene are a frequent cause of autosomal dominant progressive cochleo-vestibular dysfunction, but not of Meniere's disease. Eur J Hum Genet 2003;11:744-8.
4. Jones SM, Robertson NG, Given S, Giersch AB, Liberman MC, Morton CC. Hearing and vestibular deficits in the Coch(-/-) null mouse model: Comparison to the Coch (G88E/G88E) mouse and to DFNA9 hearing and balance disorder. Hear Res 2011;272:42-8.
5. Hildebrand MS, Gandolfo L, Shearer AE, Webster JA, Jensen M, Kimberling WJ, et al. A novel mutation in COCH-implications for genotype-phenotype correlations in DFNA9 hearing loss. Laryngoscope 2010;120:2489-93.
6. Bom SJ, Kemperman MH, De Kok YJ, Huygen PL, Verhagen WI, Cremers FP, et al. Progressive cochleovestibular impairment caused by a point mutation in the COCH gene at DFNA9. Laryngoscope 1999;109:1525-30.
7. Baek MJ, Park HM, Johnson JM, Altuntas CZ, Jane-Wit D, Jaini R, et al. Increased frequencies of cochlin-specific T cells in patients with autoimmune sensorineural hearing loss. J Immunol 2006;177:4203-10.
8. Bhattacharya SK, Rockwood EJ, Smith SD, Bonilha VL, Crabb JS, Kuchtey RW, et al. Proteomics reveal Cochlin deposits associated with glaucomatous trabecular meshwork. J Biol Chem 2005;280:6080-4.
9. Ikezono T, Omori A, Ichinose S, Pawankar R, Watanabe A, Yagi T. Identification of the protein product of the Coch gene (hereditary deafness gene) as the major component of bovine inner ear protein. Biochim Biophys Acta 2001;1535: 258-65.
10. Ikezono T, Shindo S, Li L, Omori A, Ichinose S, Watanabe A, et al. Identification of a novel Cochlin isoform in the perilymph: Insights to Cochlin function and the pathogenesis of DFNA9. Biochem Biophys Res Commun 2004; 314:440-6.
11. Shindo S, Ikezono T, Ishizaki M, Sekiguchi S, Mizuta K, Li L, et al. Spatiotemporal expression of cochlin in the inner ear of rats during postnatal development. Neurosci Lett 2008;444:148-52.
12. Chance MR, Chang J, Liu S, Gokulrangan G, Chen DH, Lindsay A, et al. Proteomics, bioinformatics and targeted gene expression analysis reveals up-regulation of cochlin and identifies other potential biomarkers in the mouse model for deafness in Usher syndrome type 1F. Hum Mol Genet 2010; 19:1515-27.
13. Sekine K, Ikezono T, Matsumura T, Shindo S, Watanabe A, Li L, et al. Expression of cochlin mRNA splice variants in the inner ear. Audiol Neurootol 2010;15:88-96.
14. Schmittgen TD, Livak KJ. Analyzing real-time PCR data by the comparative C(T) method. Nat Protoc 2008;3: 1101-8.
15. Livak KJ, Schmittgen TD. Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) Method. Methods 2001;25:402-8.
16. Robertson NG, Cremers CW, Huygen PL, Ikezono T, Krastins B, Kremer H, et al. Cochlin immunostaining of inner ear pathologic deposits and proteomic analysis in DFNA9 deafness and vestibular dysfunction. Hum Mol Genet 2006;15:1071-85.
17. Kommareddi PK, Nair TS, Raphael Y, Telian SA, Kim AH, Arts HA, et al. Cochlin isoforms and their interaction with CTL2 (SLC44A2) in the inner ear. J Assoc Res Otolaryngol 2007;8:435-46.
18. Robertson NG, Skvorak AB, Yin Y, Weremowicz S, Johnson KR, Kovatch KA, et al. Mapping and characterization of a novel cochlear gene in human and in mouse: A positional candidate gene for a deafness disorder, DFNA9. Genomics 1997;46:345-54.
19. Robertson NG, Hamaker SA, Patriub V, Aster JC, Morton CC. Subcellular localisation, secretion, and posttranslational processing of normal cochlin, and of mutants causing the sensorineural deafness and vestibular disorder, DFNA9. J Med Genet 2003;40:479-86.
20. Yao J, Py BF, Zhu H, Bao J, Yuan J. Role of protein misfolding in DFNA9 hearing loss. J Biol Chem 2010;285: 14909-19.