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Coordinated control of connexin 26 and connexin 30 at the regulatory and functional level in the inner ear.

Ortolano S, Di Pasquale G, Crispino G, Anselmi F, Mammano F, Chiorini JA.

Proc Natl Acad Sci U S A. 2008 Dec 2;105(48):18776-81. doi: 10.1073/pnas.0800831105. Epub 2008 Dec 1.


The connexin 30.3 of zebrafish homologue of human connexin 26 may play similar role in the inner ear.

Chang-Chien J, Yen YC, Chien KH, Li SY, Hsu TC, Yang JJ.

Hear Res. 2014 Jul;313:55-66. doi: 10.1016/j.heares.2014.04.010. Epub 2014 May 6.


Hearing is normal without connexin30.

Boulay AC, del Castillo FJ, Giraudet F, Hamard G, Giaume C, Petit C, Avan P, Cohen-Salmon M.

J Neurosci. 2013 Jan 9;33(2):430-4. doi: 10.1523/JNEUROSCI.4240-12.2013.


The human deafness-associated connexin 30 T5M mutation causes mild hearing loss and reduces biochemical coupling among cochlear non-sensory cells in knock-in mice.

Schütz M, Scimemi P, Majumder P, De Siati RD, Crispino G, Rodriguez L, Bortolozzi M, Santarelli R, Seydel A, Sonntag S, Ingham N, Steel KP, Willecke K, Mammano F.

Hum Mol Genet. 2010 Dec 15;19(24):4759-73. doi: 10.1093/hmg/ddq402. Epub 2010 Sep 21.


ATP release through connexin hemichannels and gap junction transfer of second messengers propagate Ca2+ signals across the inner ear.

Anselmi F, Hernandez VH, Crispino G, Seydel A, Ortolano S, Roper SD, Kessaris N, Richardson W, Rickheit G, Filippov MA, Monyer H, Mammano F.

Proc Natl Acad Sci U S A. 2008 Dec 2;105(48):18770-5. doi: 10.1073/pnas.0800793105. Epub 2008 Dec 1.


BAAV mediated GJB2 gene transfer restores gap junction coupling in cochlear organotypic cultures from deaf Cx26Sox10Cre mice.

Crispino G, Di Pasquale G, Scimemi P, Rodriguez L, Galindo Ramirez F, De Siati RD, Santarelli RM, Arslan E, Bortolozzi M, Chiorini JA, Mammano F.

PLoS One. 2011;6(8):e23279. doi: 10.1371/journal.pone.0023279. Epub 2011 Aug 18.


Gap junctions in the inner ear: comparison of distribution patterns in different vertebrates and assessement of connexin composition in mammals.

Forge A, Becker D, Casalotti S, Edwards J, Marziano N, Nevill G.

J Comp Neurol. 2003 Dec 8;467(2):207-31.


Mutations in the gene for connexin 26 (GJB2) that cause hearing loss have a dominant negative effect on connexin 30.

Marziano NK, Casalotti SO, Portelli AE, Becker DL, Forge A.

Hum Mol Genet. 2003 Apr 15;12(8):805-12.


Unique expression of connexins in the human cochlea.

Liu W, Boström M, Kinnefors A, Rask-Andersen H.

Hear Res. 2009 Apr;250(1-2):55-62. doi: 10.1016/j.heares.2009.01.010. Epub 2009 Feb 6.


Connexin32 can restore hearing in connexin26 deficient mice.

Degen J, Schütz M, Dicke N, Strenzke N, Jokwitz M, Moser T, Willecke K.

Eur J Cell Biol. 2011 Oct;90(10):817-24. doi: 10.1016/j.ejcb.2011.05.001. Epub 2011 Aug 2.


Cochlear gap junctions coassembled from Cx26 and 30 show faster intercellular Ca2+ signaling than homomeric counterparts.

Sun J, Ahmad S, Chen S, Tang W, Zhang Y, Chen P, Lin X.

Am J Physiol Cell Physiol. 2005 Mar;288(3):C613-23.


Gap junctions and connexins in the inner ear: their roles in homeostasis and deafness.

Nickel R, Forge A.

Curr Opin Otolaryngol Head Neck Surg. 2008 Oct;16(5):452-7. doi: 10.1097/MOO.0b013e32830e20b0. Review.


Intracellular domains of mouse connexin26 and -30 affect diffusional and electrical properties of gap junction channels.

Manthey D, Banach K, Desplantez T, Lee CG, Kozak CA, Traub O, Weingart R, Willecke K.

J Membr Biol. 2001 May 15;181(2):137-48.


Functional studies of human skin disease- and deafness-associated connexin 30 mutations.

Common JE, Becker D, Di WL, Leigh IM, O'Toole EA, Kelsell DP.

Biochem Biophys Res Commun. 2002 Nov 15;298(5):651-6.


Analysis of connexin subunits required for the survival of vestibular hair cells.

Qu Y, Tang W, Dahlke I, Ding D, Salvi R, Söhl G, Willecke K, Chen P, Lin X.

J Comp Neurol. 2007 Oct 10;504(5):499-507.


[Functional interaction of the C-terminal of Nogo protein with connexin 26 and the expression of Nogo's mRNA in the murine inner ear].

Xiao ZA, Xie DH, Hu P, Xia K, Cai F, Pan Q.

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2006 Oct;23(5):492-6. Chinese.


Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness.

Ahmad S, Tang W, Chang Q, Qu Y, Hibshman J, Li Y, Söhl G, Willecke K, Chen P, Lin X.

Proc Natl Acad Sci U S A. 2007 Jan 23;104(4):1337-41. Epub 2007 Jan 16.


Assembly of the cochlear gap junction macromolecular complex requires connexin 26.

Kamiya K, Yum SW, Kurebayashi N, Muraki M, Ogawa K, Karasawa K, Miwa A, Guo X, Gotoh S, Sugitani Y, Yamanaka H, Ito-Kawashima S, Iizuka T, Sakurai T, Noda T, Minowa O, Ikeda K.

J Clin Invest. 2014 Apr;124(4):1598-607. doi: 10.1172/JCI67621. Epub 2014 Mar 3.

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