Dominant negative connexin26 mutation R75W causing severe hearing loss influences normal programmed cell death in postnatal organ of Corti

BMC Genetics

Volumn 15, Issue , 2014, Pages

  Inoshita, Ayako ^a   Karasawa, Keiko ^a   Funakubo, Megumi ^a   Miwa, Asuka ^a   Ikeda, Katsuhisa ^a   Kamiya, Kazusaku ^a  

^a JUNTENDO UNIVERSITY SCHOOL OF MEDICINE   (Japan)

Author keywords

Apoptosis; Gjb2; Greater epithelial ridge; Hereditary hearing loss; Mouse; Organ of corti

Indexed keywords

CONNEXIN 26;

ANIMAL EXPERIMENT; ANIMAL TISSUE; APOPTOSIS; ARTICLE; CELL PROLIFERATION; COCHLEA; CONTROLLED STUDY; CORTI ORGAN; DISEASE ASSOCIATION; DISEASE SEVERITY; EMBRYO DEVELOPMENT; GENE MUTATION; HEARING IMPAIRMENT; IMMUNOHISTOCHEMISTRY; MICROSCOPY; MORPHOLOGY; MOUSE; NONHUMAN; PERINATAL PERIOD; TRANSMISSION ELECTRON MICROSCOPY;

ANIMALS; APOPTOSIS; CASPASE 3; CONNEXINS; HEARING LOSS; MICE; MICE, INBRED C57BL; MICE, TRANSGENIC; MUTATION; ORGAN OF CORTI;

EID: 84892512877     PISSN: None     EISSN: 14712156     Source Type: Journal    
DOI: 10.1186/1471-2156-15-1     Document Type: Article

References (32)

1. Dere E, Zlomuzica A. The role of gap junctions in the brain in health and disease. Neurosci Biobehav Rev 2012, 36(1):206-217. 10.1016/j.neubiorev.2011.05.015, 21664373.
2. Elias LA, Wang DD, Kriegstein AR. Gap junction adhesion is necessary for radial migration in the neocortex. Nature 2007, 448(7156):901-907. 10.1038/nature06063, 17713529.
3. Loiselle AE, Jiang JX, Donahue HJ. Gap junction and hemichannel functions in osteocytes. Bone 2013, 54(2):205-212. 10.1016/j.bone.2012.08.132, 23069374.
4. Vinken M. Gap junctions and non-neoplastic liver disease. J Hepatol 2012, 57(3):655-662. 10.1016/j.jhep.2012.02.036, 22609308.
5. Xu J, Nicholson BJ. The role of connexins in ear and skin physiology - functional insights from disease-associated mutations. Biochim Biophys Acta 2013, 1828(1):167-178. 10.1016/j.bbamem.2012.06.024, 3521577, 22796187.
6. Inoshita A, Iizuka T, Okamura HO, Minekawa A, Kojima K, Furukawa M, Kusunoki T, Ikeda K. Postnatal development of the organ of Corti in dominant-negative Gjb2 transgenic mice. Neuroscience 2008, 156(4):1039-1047. 10.1016/j.neuroscience.2008.08.027, 18793701.
7. Kudo T, Kure S, Ikeda K, Xia AP, Katori Y, Suzuki M, Kojima K, Ichinohe A, Suzuki Y, Aoki Y, 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(9):995-1004. 10.1093/hmg/ddg116, 12700168.
8. Minekawa A, Abe T, Inoshita A, Iizuka T, Kakehata S, Narui Y, Koike T, Kamiya K, Okamura HO, Shinkawa H, et al. Cochlear outer hair cells in a dominant-negative connexin26 mutant mouse preserve non-linear capacitance in spite of impaired distortion product otoacoustic emission. Neuroscience 2009, 164(3):1312-1319. 10.1016/j.neuroscience.2009.08.043, 19712724.
9. Anniko M. Postnatal maturation of cochlear sensory hairs in the mouse. Anat Embryol (Berl) 1983, 166(3):355-368. 10.1007/BF00305923, 6869851.
10. Zheng JL, Gao WQ. Overexpression of Math1 induces robust production of extra hair cells in postnatal rat inner ears. Nat Neurosci 2000, 3(6):580-586. 10.1038/75753, 10816314.
11. Nishizaki K, Anniko M, Orita Y, Masuda Y, Yoshino T, Kanda S, Sasaki J. Programmed cell death in the development of the mouse external auditory canal. Anat Rec 1998, 252(3):378-382. 10.1002/(SICI)1097-0185(199811)252:3<378::AID-AR5>3.0.CO;2-#, 9811215.
12. Rueda J, de la Sen C, Juiz JM, Merchan JA. Neuronal loss in the spiral ganglion of young rats. Acta Otolaryngol 1987, 104(5-6):417-421.
13. Kamiya K, Takahashi K, Kitamura K, Momoi T, Yoshikawa Y. Mitosis and apoptosis in postnatal auditory system of the C3H/He strain. Brain Res 2001, 901(1-2):296-302.
14. Nagahara A, Watanabe S, Miwa H, Endo K, Hirose M, Sato N. Reduction of gap junction protein connexin 32 in rat atrophic gastric mucosa as an early event in carcinogenesis. J Gastroenterol 1996, 31(4):491-497. 10.1007/BF02355047, 8844468.
15. Tsai H, Werber J, Davia MO, Edelman M, Tanaka KE, Melman A, Christ GJ, Geliebter J. Reduced connexin 43 expression in high grade, human prostatic adenocarcinoma cells. Biochem Biophys Res Commun 1996, 227(1):64-69. 10.1006/bbrc.1996.1468, 8858104.
16. Huang RP, Hossain MZ, Sehgal A, Boynton AL. Reduced connexin43 expression in high-grade human brain glioma cells. J Surg Oncol 1999, 70(1):21-24. 10.1002/(SICI)1096-9098(199901)70:1<21::AID-JSO4>3.0.CO;2-0, 9989416.
17. Laird DW, Fistouris P, Batist G, Alpert L, Huynh HT, Carystinos GD, Alaoui-Jamali MA. Deficiency of connexin43 gap junctions is an independent marker for breast tumors. Cancer Res 1999, 59(16):4104-4110.
18. Jinn Y, Ichioka M, Marumo F. Expression of connexin32 and connexin43 gap junction proteins and E-cadherin in human lung cancer. Cancer lett 1998, 127(1-2):161-169.
19. Eghbali B, Kessler JA, Reid LM, Roy C, Spray DC. Involvement of gap junctions in tumorigenesis: transfection of tumor cells with connexin 32 cDNA retards growth in vivo. Proc Natl Acad Sci USA 1991, 88(23):10701-10705. 10.1073/pnas.88.23.10701, 52998, 1660148.
20. Huang RP, Fan Y, Hossain MZ, Peng A, Zeng ZL, Boynton AL. Reversion of the neoplastic phenotype of human glioblastoma cells by connexin 43 (cx43). Cancer Res 1998, 58(22):5089-5096.
21. Trosko JE, Ruch RJ. Cell-cell communication in carcinogenesis. Front Biosci 1998, 3:d208-236.
22. Hellmann P, Grummer R, Schirrmacher K, Rook M, Traub O, Winterhager E. Transfection with different connexin genes alters growth and differentiation of human choriocarcinoma cells. Exp Cell Res 1999, 246(2):480-490. 10.1006/excr.1998.4332, 9925764.
23. Lee HJ, Lee IK, Seul KH, Rhee SK. Growth inhibition by connexin26 expression in cultured rodent tumor cells. Mol Cells 2002, 14(1):136-142.
24. Momiyama M, Omori Y, Ishizaki Y, Nishikawa Y, Tokairin T, Ogawa J, Enomoto K. Connexin26-mediated gap junctional communication reverses the malignant phenotype of MCF-7 breast cancer cells. Cancer Sci 2003, 94(6):501-507. 10.1111/j.1349-7006.2003.tb01473.x, 12824874.
25. Huang RP, Hossain MZ, Huang R, Gano J, Fan Y, Boynton AL. Connexin 43 (cx43) enhances chemotherapy-induced apoptosis in human glioblastoma cells. Int J Cancer J Int Cancer 2001, 92(1):130-138.
26. Tanaka M, Grossman HB. Connexin 26 induces growth suppression, apoptosis and increased efficacy of doxorubicin in prostate cancer cells. Oncol Rep 2004, 11(2):537-541.
27. Kanczuga-Koda L, Sulkowski S, Tomaszewski J, Koda M, Sulkowska M, Przystupa W, Golaszewska J, Baltaziak M. Connexins 26 and 43 correlate with Bak, but not with Bcl-2 protein in breast cancer. Oncol Rep 2005, 14(2):325-329.
28. Trosko JE, Ruch RJ. Gap junctions as targets for cancer chemoprevention and chemotherapy. Curr Drug Targets 2002, 3(6):465-482. 10.2174/1389450023347371, 12448698.
29. Chen Y, Huhn D, Knosel T, Pacyna-Gengelbach M, Deutschmann N, Petersen I. Downregulation of connexin 26 in human lung cancer is related to promoter methylation. Int J Cancer J Int Cancer 2005, 113(1):14-21.
30. Tan LW, Bianco T, Dobrovic A. Variable promoter region CpG island methylation of the putative tumor suppressor gene Connexin 26 in breast cancer. Carcinogenesis 2002, 23(2):231-236. 10.1093/carcin/23.2.231, 11872627.
31. Souter M, Nevill G, Forge A. Postnatal maturation of the organ of Corti in gerbils: morphology and physiological responses. J Comp Neurol 1997, 386(4):635-651. 10.1002/(SICI)1096-9861(19971006)386:4<635::AID-CNE9>3.0.CO;2-3, 9378857.
32. Takahashi K, Kamiya K, Urase K, Suga M, Takizawa T, Mori H, Yoshikawa Y, Ichimura K, Kuida K, Momoi T. Caspase-3-deficiency induces hyperplasia of supporting cells and degeneration of sensory cells resulting in the hearing loss. Brain Res 2001, 894(2):359-367. 10.1016/S0006-8993(01)02123-0, 11251216.