Early developmental expression of connexin26 in the cochlea contributes to its dominate functional role in the cochlear gap junctions

Biochemical and Biophysical Research Communications

Volumn 417, Issue 1, 2012, Pages 245-250

  Qu, Yan ^a   Tang, Wenxue ^b   Zhou, Binfei ^b   Ahmad, Shoeb ^b   Chang, Qing ^b   Li, Xiaoming ^a   Lin, Xi ^b  

^a HEBEI MEDICAL UNIVERSITY   (China)

^b EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Connexin26; Connexin30; Functional studies; Gap junctions; Hearing rescue; Hereditary deafness; Mouse model

Indexed keywords

CONNEXIN 26; CONNEXIN 30;

ANIMAL EXPERIMENT; ANTIBODY LABELING; ARTICLE; BACTERIAL ARTIFICIAL CHROMOSOME; COCHLEA; CONTROLLED STUDY; CORTI ORGAN; EVOKED BRAIN STEM AUDITORY RESPONSE; GAP JUNCTION; GENE DELETION; GENE OVEREXPRESSION; HEARING; MORPHOLOGY; MOUSE; NONHUMAN; POSTNATAL DEVELOPMENT; PRIORITY JOURNAL; PROTEIN ASSEMBLY; PROTEIN EXPRESSION; PROTEIN FUNCTION; TRANSGENIC MOUSE;

ANIMALS; CHROMOSOMES, ARTIFICIAL, BACTERIAL; COCHLEA; CONNEXINS; GAP JUNCTIONS; GENE DELETION; HEARING LOSS; HUMANS; MICE; MICE, TRANSGENIC;

BACTERIA (MICROORGANISMS); MUS;

EID: 84855794365     PISSN: 0006291X     EISSN: 10902104     Source Type: Journal    
DOI: 10.1016/j.bbrc.2011.11.093     Document Type: Article

References (23)

1. Kelsell D.P., Dunlop J., Stevens H.P., Lench N.J., Liang J.N., Parry G., Mueller R.F., Leigh I.M. Connexin 26 mutations in hereditary non-syndromic sensorineural deafness. Nature 1997, 387:80-83.
2. Estivill X., Fortina P., Surrey S., Rabionet R., Melchionda S., D'Agruma L., Mansfield E., Rappaport E., Govea N., Mila M., Zelante L., Gasparini P. Connexin-26 mutations in sporadic and inherited sensorineural deafness. Lancet 1998, 351:394-398.
3. Alexander D.B., Goldberg G.S. Transfer of biologically important molecules between cells through gap junction channels. Curr. Med. Chem. 2003, 10:2045-2058.
4. Guilford P., Ben Arab S., Blanchard S., Levilliers J., Weissenbach J., Belkahia A., Petit C. A non-syndrome form of neurosensory, recessive deafness maps to the pericentromeric region of chromosome 13q. Nat. Genet. 1994, 6:24-28.
5. Grifa A., Wagner C.A., D'Ambrosio L., Melchionda S., Bernardi F., Lopez-Bigas N., Rabionet R., Arbones M., Monica M.D., Estivill X., Zelante L., Lang F., Gasparini P. Mutations in GJB6 cause nonsyndromic autosomal dominant deafness at DFNA3 locus. Nat. Genet. 1999, 23:16-18.
6. del Castillo I., Villamar M., Moreno-Pelayo M.A., del Castillo F.J., Alvarez A., Telleria D., Menendez I., Moreno F. A deletion involving the connexin 30 gene in nonsyndromic hearing impairment. N. Engl. J. Med. 2002, 346:243-249.
7. Liu X.Z., Xia X.J., Ke X.M., Ouyang X.M., Du L.L., Liu Y.H., Angeli S., Telischi F.F., Nance W.E., Balkany T., Xu L.R. The prevalence of connexin 26 (GJB2) mutations in the Chinese population. Hum. Genet. 2002, 111:394-397.
8. Denoyelle F., Weil D., Maw M.A., Wilcox S.A., Lench N.J., Allen-Powell D.R., Osborn A.H., Dahl H.H., Middleton A., Houseman M.J., Dode C., Marlin S., Boulila-ElGaied A., Grati M., Ayadi H., BenArab S., Bitoun P., Lina-Granade G., Godet J., Mustapha M., Loiselet J., El-Zir E., Aubois A., Joannard A., Petit C., et al. Prelingual deafness: high prevalence of a 30delG mutation in the connexin 26 gene. Hum. Mol. Genet. 1997, 6:2173-2177.
9. Hoang Dinh E., Ahmad S., Chang Q., Tang W., Stong B., Lin X. Diverse deafness mechanisms of connexin mutations revealed by studies using in vitro approaches and mouse models. Brain Res. 2009.
10. Cohen-Salmon M., Ott T., Michel V., Hardelin J.P., Perfettini I., Eybalin M., Wu T., Marcus D.C., Wangemann P., Willecke K., Petit C. Targeted ablation of connexin26 in the inner ear epithelial gap junction network causes hearing impairment and cell death. Curr. Biol. 2002, 12:1106-1111.
11. Wang Y., Chang Q., Tang W., Sun Y., Zhou B., Li H., Lin X. Targeted connexin26 ablation arrests postnatal development of the organ of Corti. Biochem. Biophys. Res. Commun. 2009, 385:33-37.
12. Teubner B., Michel V., Pesch J., Lautermann J., Cohen-Salmon M., Sohl G., Jahnke K., Winterhager E., Herberhold C., Hardelin J.P., Petit C., Willecke K. Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential. Hum. Mol. Genet. 2003, 12:13-21.
13. Lautermann J., ten Cate W.J., Altenhoff P., Grummer R., Traub O., Frank H., Jahnke K., Winterhager E. Expression of the gap-junction connexins 26 and 30 in the rat cochlea. Cell Tissue Res. 1998, 294:415-420.
14. Ahmad S., Chen S., Sun J., Lin X. Connexins 26 and 30 are co-assembled to form gap junctions in the cochlea of mice. Biochem. Biophys. Res. Commun. 2003, 307:362-368.
15. Forge A., Becker D., Casalotti S., Edwards J., Marziano N., Nevill G. Gap junctions in the inner ear: comparison of distribution patterns in different vertebrates and assessement of connexin composition in mammals. J. Comp. Neurol. 2003, 467:207-231.
16. Ahmad S., Tang W., Chang Q., Qu Y., Hibshman J., Li Y., Sohl G., Willecke K., Chen P., Lin X. Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness. Proc. Natl. Acad. Sci. U. S. A. 2007, 104:1337-1341.
17. Sun J., Ahmad S., Chen S., Tang W., Zhang Y., Chen P., Lin X. Cochlear gap junctions coassembled from Cx26 and 30 show faster intercellular Ca2+ signaling than homomeric counterparts. Am. J. Physiol. Cell Physiol. 2005, 288:C613-623.
18. Sun Y., Tang W., Chang Q., Wang Y.F., Kong Y.Y., Lin X. Connexin30 null and conditional connexin26 null mice display distinct pattern and time course of cellular degeneration in the cochlea. J. Comp. Neurol. 2009, 516:569-579.
19. Wilch E., Zhu M., Burkhart K.B., Regier M., Elfenbein J.L., Fisher R.A., Friderici K.H. Expression of GJB2 and GJB6 is reduced in a novel DFNB1 allele. Am. J. Hum. Genet. 2006, 79:174-179.
20. Pujol R., Lavigne-Rebillard M., Lenoir M. Development of sensory and neural structures in the mammalian cochlea. Development of the Auditory System 1998, 146-192. Springer, Berlin, New York. E.W. Rubel, A.N. Popper, R.R. Fay (Eds.).
21. Wangemann P. K+ cycling and the endocochlear potential. Hear. Res. 2002, 165:1-9.
22. Kikuchi T., Adams J., Miyabe Y., So E., Kobayashi T. Potassium ion recycling pathway via gap junction systems in the mammalian cochlea and its interruption in hereditary nonsyndromic deafness. Med. Electron Microsc. 2000, 33:p51-56.
23. Chang Q., Tang W., Ahmad S., Zhou B., Lin X. Gap junction mediated intercellular metabolite transfer in the cochlea is compromised in connexin30 null mice. PLoS One 2008, 3:e4088.