Qiu Y, Zheng J, Chen S, Sun Y. Connexin mutations and hereditary diseases. Int J Mol Sci 2022, 23: 4255.
Article CAS PubMed PubMed Central Google Scholar
Hosoya M, Fujioka M, Murayama AY, Ogawa K, Okano H, Ozawa H. Dynamic spatiotemporal expression changes in connexins of the developing primate’s cochlea. Genes (Basel) 2021, 12: 1082.
Article CAS PubMed Google Scholar
Lautermann J, ten Cate WJF, Altenhoff P, Grümmer R, Traub O, Frank HG. Expression of the gap-junction connexins 26 and 30 in the rat cochlea. Cell Tissue Res 1998, 294: 415–420.
Article CAS PubMed Google Scholar
Liu W, Li H, Edin F, Brännström J, Glueckert R, Schrott-Fischer A, et al. Molecular composition and distribution of gap junctions in the sensory epithelium of the human cochlea-a super-resolution structured illumination microscopy (SR-SIM) study. Ups J Med Sci 2017, 122: 160–170.
Article PubMed PubMed Central Google Scholar
Liu W, Edin F, Blom H, Magnusson P, Schrott-Fischer A, Glueckert R, et al. Super-resolution structured illumination fluorescence microscopy of the lateral wall of the cochlea: the Connexin26/30 proteins are separately expressed in man. Cell Tissue Res 2016, 365: 13–27.
Article CAS PubMed Google Scholar
Kenna MA, Feldman HA, Neault MW, Frangulov A, Wu BL, Fligor B, et al. Audiologic phenotype and progression in GJB2 (Connexin 26) hearing loss. Arch Otolaryngol Head Neck Surg 2010, 136: 81–87.
Article PubMed PubMed Central Google Scholar
Shearer AE, Hildebrand MS, Smith RJ. Hereditary hearing loss and deafness overview. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJ, Stephens K, editors. Gene Review. Seattle (WA): University of Washington; 1993–2018.
Takada Y, Beyer LA, Swiderski DL, O’Neal AL, Prieskorn DM, Shivatzki S, et al. Connexin 26 null mice exhibit spiral ganglion degeneration that can be blocked by BDNF gene therapy. Hear Res 2014, 309: 124–135.
Article CAS PubMed Google Scholar
Shearer AE, Eppsteiner RW, Frees K, Tejani V, Sloan-Heggen CM, Brown C, et al. Genetic variants in the peripheral auditory system significantly affect adult cochlear implant performance. Hear Res 2017, 348: 138–142.
Article PubMed PubMed Central Google Scholar
Wu CM, Ko HC, Tsou YT, Lin YH, Lin JL, Chen CK, et al. Long-term cochlear implant outcomes in children with GJB2 and SLC26A4 mutations. PLoS One 2015, 10: e0138575.
Article PubMed PubMed Central Google Scholar
Barclay M, Ryan AF, Housley GD. Type I vs type II spiral ganglion neurons exhibit differential survival and neuritogenesis during cochlear development. Neural Dev 2011, 6: 33.
Article CAS PubMed PubMed Central Google Scholar
Huang LC, Thorne PR, Housley GD, Montgomery JM. Spatiotemporal definition of neurite outgrowth, refinement and retraction in the developing mouse cochlea. Development 2007, 134: 2925–2933.
Article CAS PubMed Google Scholar
Rubel EW, Fritzsch B. Auditory system development: Primary auditory neurons and their targets. Annu Rev Neurosci 2002, 25: 51–101.
Article CAS PubMed Google Scholar
Panganiban CH, Barth JL, Darbelli L, Xing Y, Zhang J, Li H, et al. Noise-induced dysregulation of Quaking RNA binding proteins contributes to auditory nerve demyelination and hearing loss. J Neurosci 2018, 38: 2551–2568.
Article CAS PubMed PubMed Central Google Scholar
Wan G, Corfas G. Transient auditory nerve demyelination as a new mechanism for hidden hearing loss. Nat Commun 2017, 8: 14487.
Article ADS CAS PubMed PubMed Central Google Scholar
Qing Chang. Timed conditional null of connexin26 in mice reveals temporary requirements of connexin26 in key cochlear developmental events before the onset of hearing. Neurobiol Dis 2015, 73: 418–427.
Chen S, Sun Y, Lin X, Kong W. Down regulated connexin26 at different postnatal stage displayed different types of cellular degeneration and formation of organ of Corti. Biochem Biophys Res Commun 2014, 445: 71–77.
Article CAS PubMed Google Scholar
Kudo T, Kure S, Ikeda K, Xia AP, Katori Y, Suzuki M, et al. Transgenic expression of a dominant-negative connexin26 causes degeneration of the organ of Corti and non-syndromic deafness. Hum Mol Genet 2003, 12: 995–1004.
Article CAS PubMed Google Scholar
Chen S, Xie L, Xu K, Cao HY, Wu X, Xu XX, et al. Developmental abnormalities in supporting cell phalangeal processes and cytoskeleton in the Gjb2 knockdown mouse model. Dis Model Mech 2018, 11: dmm033019.
Article PubMed PubMed Central Google Scholar
Liu XZ, Jin Y, Chen S, Xu K, Xie L, Qiu Y, et al. F-actin dysplasia involved in organ of corti deformity in Gjb2 knockdown mouse model. Front Mol Neurosci 2021, 14: 808553.
Article CAS PubMed Google Scholar
Amor V, Feinberg K, Eshed-Eisenbach Y, Vainshtein A, Frechter S, Grumet M, et al. Long-term maintenance of Na+ channels at nodes of Ranvier depends on glial contact mediated by gliomedin and NrCAM. J Neurosci 2014, 34: 5089–5098.
Article PubMed PubMed Central Google Scholar
Chen P, Wu W, Zhang J, Chen J, Li Y, Sun L, et al. Pathological mechanisms of connexin26-related hearing loss: Potassium recycling, ATP-calcium signaling, or energy supply? Front Mol Neurosci 2022, 15: 976388.
Article CAS PubMed PubMed Central Google Scholar
Walters BJ, Yamashita T, Zuo J. Sox2-CreER mice are useful for fate mapping of mature, but not neonatal, cochlear supporting cells in hair cell regeneration studies. Sci Rep 2015, 5: 11621.
Article ADS CAS PubMed PubMed Central Google Scholar
Zhu Y, Liang C, Chen J, Zong L, Chen GD, Zhao HB. Active cochlear amplification is dependent on supporting cell gap junctions. Nat Commun 2013, 4: 1786.
Article ADS PubMed Google Scholar
Froud KE, Wong AC, Cederholm JM, Klugmann M, Sandow SL, Julien JP, et al. Type II spiral ganglion afferent neurons drive medial olivocochlear reflex suppression of the cochlear amplifier. Nat Commun 2015, 6: 7115.
留言 (0)