Deafness induced by Connexin 26 (GJB2) deficiency is not determined by endocochlear potential (EP) reduction but is associated with cochlear developmental disorders

Biochemical and Biophysical Research Communications

Volumn 448, Issue 1, 2014, Pages 28-32

  Chen, Jin ^a,b   Chen, Jing ^a   Zhu, Yan ^a   Liang, Chun ^a   Zhao, Hong Bo ^a  

^a UNIVERSITY OF KENTUCKY   (United States)

^b HUAZHONG UNIVERSITY OF SCIENCE AND TECHNOLOGY   (China)

Author keywords

Connexin; Cx26; Deafness; EP; Gap junction; Inner ear development

Indexed keywords

CONNEXIN 26;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; AUDITORY THRESHOLD; COCHLEA; COCHLEA POTENTIAL; COCHLEAR DEVELOPMENT; CONGENITAL DEAFNESS; CONTROLLED STUDY; DEVELOPMENTAL DISORDER; EVOKED BRAIN STEM AUDITORY RESPONSE; KNOCKOUT MOUSE; MOUSE; NEWBORN; NONHUMAN; ORGANOGENESIS; POSTNATAL DEVELOPMENT; PRIORITY JOURNAL;

MUS;

CONNEXIN; CX26; DEAFNESS; EP; GAP JUNCTION; INNER EAR DEVELOPMENT;

ANIMALS; ANIMALS, NEWBORN; COCHLEA; CONNEXINS; DEAFNESS; MICE; MICE, KNOCKOUT;

EID: 84901191078     PISSN: 0006291X     EISSN: 10902104     Source Type: Journal    
DOI: 10.1016/j.bbrc.2014.04.016     Document Type: Article

References (22)

1. J.A. Mason, and K.R. Herrmann Universal infant hearing screening by automated auditory brainstem response measurement Pediatrics 101 1998 221 228
2. M.B. Petersen, and P.J. Willems Non-syndromic, autosomal-recessive deafness Clin. Genet. 69 2006 371 392
3. H.B. Zhao, T. Kikuchi, A. Ngezahayo, and T.W. White Gap junctions and cochlear homeostasis J. Membr. Biol. 209 2006 177 186
4. R. Dobrowolski, and K. Willecke Connexin-caused genetic diseases and corresponding mouse models Antioxid. Redox Signal. 11 2009 283 295
5. D.P. Kelsell, J. Dunlop, H.P. Stevens, N.J. Lench, J.N. Liang, G. Parry, R.F. Mueller, and I.M. Leigh Connexin 26 mutations in hereditary non-syndromic sensorineural deafness Nature 387 1997 80 83
6. F.J. Castillo, and I. Castillo The DFNB1 subtype of autosomal recessive non-syndromic hearing impairment Front. Biosci. 17 2011 3252 3274
7. M. Cohen-Salmon, T. Ott, V. Michel, J.P. Hardelin, I. Perfettini, M. Eybalin, T. Wu, D.C. Marcus, P. Wangemann, K. Willecke, and C. Petit Targeted ablation of connexin26 in the inner ear epithelial gap junction network causes hearing impairment and cell death Curr. Biol. 12 2002 1106 1111
8. Y. Sun, W. Tang, Q. Chang, Y. Wang, W. Kong, and X. Lin Connexin30 null and conditional connexin26 null mice display distinct pattern and time course of cellular degeneration in the cochlea J. Comp. Neurol. 516 2009 569 579
9. Y. Wang, Q. Chang, W. Tang, Y. Sun, B. Zhou, H. Li, and X. Lin Targeted connexin26 ablation arrests postnatal development of the organ of Corti Biochem. Biophys. Res. Commun. 385 2009 33 37
10. C. Liang, Y. Zhu, L. Zong, G.J. Lu, and H.B. Zhao Cell degeneration is not a primary causer for Connexin26 (GJB2) deficiency associated hearing loss Neurosci. Lett. 528 2012 36 41
11. Y. Zhu, C. Liang, J. Chen, L. Zong, G.D. Chen, and H.B. Zhao Active cochlear amplification is dependent on supporting cell gap junctions Nat. Commun. 4 2013 1786 10.1038/ncomms2806
12. P. Wangemann Supporting sensory transduction: cochlear fluid homeostasis and the endocochlear potential J. Physiol. 576 2006 11 21
13. + channel (Kir4.1) in the inner ear and hearing loss Neuroscience 265 2014 137 146
14. L.M. Chow, Y. Tian, T. Weber, M. Corbett, J. Zuo, and S.J. Baker Inducible Cre recombinase activity in mouse cerebellar granule cell precursors and inner ear hair cells Dev. Dyn. 235 2006 2991 2998
15. B. Teubner, V. Michel, J. Pesch, J. Lautermann, M. Cohen-Salmon, G. Sohl, K. Jahnke, E. Winterhager, C. Herberhold, J.P. Hardelin, C. Petit, and K. Willecke Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential Hum. Mol. Genet. 12 2003 13 21
16. H.B. Zhao, and N. Yu Distinct and gradient distributions of connexin26 and connexin30 in the cochlear sensory epithelium of guinea pigs J. Comp. Neurol. 499 2006 506 518
17. Y.P. Liu, and H.B. Zhao Cellular characterization of Connexin26 and Connnexin30 expression in the cochlear lateral wall Cell Tissue Res. 333 2008 395 403
18. A. Minekawa, T. Abe, A. Inoshita, T. Iizuka, S. Kakehata, Y. Narui, T. Koike, K. Kamiya, H.O. Okamura, H. Shinkawa, and K. Ikeda Cochlear outer hair cells in a dominant-negative connexin26 mutant mouse preserve non-linear capacitance in spite of impaired distortion product otoacoustic emission Neuroscience 164 2009 1312 1319
19. A. Inoshita, T. Iizuka, H.O. Okamura, A. Minekawa, K. Kojima, M. Furukawa, T. Kusunoki, and K. Ikeda Postnatal development of the organ of Corti in dominant-negative Gjb2 transgenic mice Neuroscience 156 2008 1039 1047
20. P. Dallos Cochlear amplification, outer hair cells and prestin Curr. Opin. Neurobiol. 18 2008 370 376
21. A.J. Hudspeth Making an effort to listen: mechanical amplification in the ear Neuron 59 2008 530 545
22. S. Chen, Y. Sun, X. Lin, and W. Kong Down regulated connexin26 at different postnatal stage displayed different types of cellular degeneration and formation of organ of Corti Biochem. Biophys. Res. Commun. 445 2014 71 77