Timed conditional null of connexin26 in mice reveals temporary requirements of connexin26 in key cochlear developmental events before the onset of hearing

Neurobiology of Disease

Volumn 73, Issue , 2015, Pages 418-427

  Chang, Qing ^a   Tang, Wenxue ^a   Kim, Yeunjung ^a   Lin, Xi ^a  

^a EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Connexin26 function; Deafness; Development; Gjb2 mutation; Potassium recycle

Indexed keywords

CONNEXIN 26; POTASSIUM; 5-BROMO-4-CHLORO-3-INDOLYL BETA-GALACTOSIDE; AMPA RECEPTOR; CTBP2 PROTEIN, MOUSE; DNA BINDING PROTEIN; FORKHEAD TRANSCRIPTION FACTOR; FOXG1 PROTEIN, MOUSE; GALACTOSIDE; GAP JUNCTION PROTEIN; GLUTAMATE RECEPTOR IONOTROPIC, AMPA 2; INDOLE DERIVATIVE; NERVE PROTEIN; PHOSPHOPROTEIN;

ADULT; AFFERENT NERVE GROUP 1; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL MEMBRANE DEPOLARIZATION; COCHLEA; COCHLEAR NERVE; CONTROLLED STUDY; CORTI ORGAN; GENE MUTATION; HEARING; HEARING IMPAIRMENT; INNER HAIR CELL; KNOCKOUT MOUSE; MOUSE; NERVE GROWTH; NEWBORN; NONHUMAN; ORGANOGENESIS; PERINATAL PERIOD; PROTEIN EXPRESSION; RIBBON SYNAPSE; SENSORY NERVE; SYNAPSE; WILD TYPE; ANIMAL; CYTOLOGY; DEFICIENCY; GAP JUNCTION; GENE EXPRESSION REGULATION; GENETICS; GROWTH, DEVELOPMENT AND AGING; HAIR CELL; METABOLISM; PATCH CLAMP TECHNIQUE; PHYSIOLOGY; SYNAPTIC TRANSMISSION; ULTRASTRUCTURE;

ANIMALS; COCHLEA; CONNEXINS; DNA-BINDING PROTEINS; FORKHEAD TRANSCRIPTION FACTORS; GALACTOSIDES; GAP JUNCTIONS; GENE EXPRESSION REGULATION, DEVELOPMENTAL; HAIR CELLS, AUDITORY, INNER; HEARING; INDOLES; MICE; MICE, KNOCKOUT; NERVE TISSUE PROTEINS; PATCH-CLAMP TECHNIQUES; PHOSPHOPROTEINS; RECEPTORS, AMPA; SYNAPTIC TRANSMISSION;

EID: 84910679532     PISSN: 09699961     EISSN: 1095953X     Source Type: Journal    
DOI: 10.1016/j.nbd.2014.09.005     Document Type: Article

References (38)

1. Ahmad S., et al. Connexins 26 and 30 are co-assembled to form gap junctions in the cochlea of mice. Biochem. Biophys. Res. Commun. 2003, 307:362-368.
2. Ahmad S., et al. 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.
3. Beltramello M., et al. Impaired permeability to Ins(1,4,5)P3 in a mutant connexin underlies recessive hereditary deafness. Nat. Cell Biol. 2005, 7:63-69.
4. Brigande J.V., Heller S. Quo vadis, hair cell regeneration?. Nat. Neurosci. 2009, 12:679-685.
5. Chang Q., et al. Gap junction mediated intercellular metabolite transfer in the cochlea is compromised in connexin30 null mice. PLoS ONE 2008, 3:e4088.
6. Chen J., et al. Deafness induced by connexin 26 (GJB2) deficiency is not determined by endocochlear potential (EP) reduction but is associated with cochlear developmental disorders. Biochem. Biophys. Res. Commun. 2014, 448:28-32.
7. Cohen-Salmon M., et al. Targeted ablation of connexin26 in the inner ear epithelial gap junction network causes hearing impairment and cell death. Curr. Biol. 2002, 12:1106-1111.
8. Cohen-Salmon M., et al. Expression of the connexin43- and connexin45-encoding genes in the developing and mature mouse inner ear. Cell Tissue Res. 2004, 316:15-22.
9. Connell S.S., et al. Performance after cochlear implantation in DFNB1 patients. Otolaryngol. Head Neck Surg. 2007, 137:596-602.
10. Dror A.A., Avraham K.B. Hearing loss: mechanisms revealed by genetics and cell biology. Annu. Rev. Genet. 2009, 43:411-437.
11. Echteler S.M., et al. Developmental alterations in the frequency map of the mammalian cochlea. Nature 1989, 341:147-149.
12. Giaume C., Venance L. Gap junctions in brain glial cells and development. Perspect. Dev. Neurobiol. 1995, 2:335-345.
13. Griffith A.J., et al. Cochleosaccular dysplasia associated with a connexin 26 mutation in keratitis-ichthyosis-deafness syndrome. Laryngoscope 2006, 116:1404-1408.
14. Hilgert N., et al. Forty-six genes causing nonsyndromic hearing impairment: which ones should be analyzed in DNA diagnostics?. Mutat. Res. 2009, 681:189-196.
15. Hoang Dinh E., et al. Diverse deafness mechanisms of connexin mutations revealed by studies using in vitro approaches and mouse models. Brain Res. 2009, 1277:52-69.
16. Huang L.C., et al. Spatiotemporal definition of neurite outgrowth, refinement and retraction in the developing mouse cochlea. Development 2007, 134:2925-2933.
17. Inoshita A., et al. Postnatal development of the organ of Corti in dominant-negative Gjb2 transgenic mice. Neuroscience 2008, 156:1039-1047.
18. Jing Z., et al. Disruption of the presynaptic cytomatrix protein bassoon degrades ribbon anchorage, multiquantal release, and sound encoding at the hair cell afferent synapse. J. Neurosci. 2013, 33:4456-4467.
19. Kelsell D.P., et al. Connexin 26 mutations in hereditary non-syndromic sensorineural deafness. Nature 1997, 387:80-83.
20. Khimich D., et al. Hair cell synaptic ribbons are essential for synchronous auditory signalling. Nature 2005, 434:889-894.
21. Kikuchi T., et al. Gap junction systems in the mammalian cochlea. Brain Res. Brain Res. Rev. 2000, 32:163-166.
22. Laird D.W. Life cycle of connexins in health and disease. Biochem. J. 2006, 394:527-543.
23. Lin X., et al. Applications of targeted gene capture and next-generation sequencing technologies in studies of human deafness and other genetic disabilities. Hear. Res. 2012, 288:67-76.
24. Maw M.A., et al. The contribution of the DFNB1 locus to neurosensory deafness in a Caucasian population. Am. J. Hum. Genet. 1995, 57:629-635.
25. Nadarajah B., Parnavelas J.G. Gap junction-mediated communication in the developing and adult cerebral cortex. Novartis Found. Symp. 1999, 219:157-170. (discussion 170-4).
26. Pujol R., et al. Development of sensory and neural structures in the mammalian cochlea. Development of the Auditory System 1998, 146-192. Springer, New York, Berlin. E.W. Rubel (Ed.).
27. Qu Y., et al. Early developmental expression of connexin26 in the cochlea contributes to its dominate functional role in the cochlear gap junctions. Biochem. Biophys. Res. Commun. 2011, 417:245-250.
28. Rubsamen R., Lippe R.W. The development of cochlear function. Development of the Auditory System 1998, 193-270. Springer, New York, Berlin. E.W. Rubel (Ed.).
29. Sohl G., Willecke K. Gap junctions and the connexin protein family. Cardiovasc. Res. 2004, 62:228-232.
30. Soriano P. Generalized lacZ expression with the ROSA26 Cre reporter strain. Nat. Genet. 1999, 21:70-71.
31. 2+ signaling than homomeric counterparts. Am. J. Physiol. Cell Physiol. 2005, 288:C613-C623.
32. Sun Y., et al. 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.
33. Tritsch N.X., et al. The origin of spontaneous activity in the developing auditory system. Nature 2007, 450:50-55.
34. Wang Y., et al. Targeted connexin26 ablation arrests postnatal development of the organ of Corti. Biochem. Biophys. Res. Commun. 2009, 385:33-37.
35. + cycling and the endocochlear potential. Hear. Res. 2002, 165:1-9.
36. Willecke K., et al. Structural and functional diversity of connexin genes in the mouse and human genome. Biol. Chem. 2002, 383:725-737.
37. Yu Q., et al. Virally-expressed connexin26 restores gap junction function in the cochlea of conditional Gjb2 knockout mice. Gene Ther. 2013, 21:71-80.
38. Zhang Y., et al. Gap junction-mediated intercellular biochemical coupling in cochlear supporting cells is required for normal cochlear functions. Proc. Natl. Acad. Sci. U. S. A. 2005, 102:15201-15206.