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Author Notes:

Correspondence: Xi Lin, PhD, Departments of Otolaryngology and Cell Biology, 615 Michael Street, Whitehead Building Room 543, Emory University School of Medicine, Atlanta, GA 30322; Telephone: 404-727-3723; Fax: 404-727-6256; Email: xlin2@emory.edu

Authors' Contributions: The first three authors contributed equally.

Subjects:

Research Funding:

This study was supported by grants to XL from NIDCD (RO1-DC006483 and R21-DC008353) and from National Natural Science Foundation of China (30728029), and also by grant supports to WT from NIDCD (R21 DC008672) and the Deafness Research Foundation.

Yunfeng Wang and Yu Sun received support from China Scholarship Council, grant #2008610021 and 200710187 respectively.

Keywords:

  • connexin26 mutation
  • cochlear development
  • cell degeneration
  • spiral ganglion neuron
  • hair cells
  • mouse models
  • genetic deafness
  • mechanism of deafness

Targeted connexin26 ablation arrests postnatal development of the organ of Corti

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Journal Title:

Biochemical and Biophysical Research Communications

Volume:

Volume 385, Number 1

Publisher:

, Pages 33-37

Type of Work:

Article | Post-print: After Peer Review

Abstract:

Mutations in the gene coding for connexin26 (Cx26) is the most common cause of human nonsyndromic hereditary deafness. To investigate deafness mechanisms underlying Cx26 null mutations, we generated three independent lines of conditional Cx26 null mice. Cell differentiation and gross cochlear morphology at birth seemed normal. However, postnatal development of the organ of Corti was stalled as the tunnel of Corti and the Nuel’s space were never opened. Cell degeneration was first observed in the Claudius cells around P8. Outer hair cell loss was initially observed around P13 at middle turn when inner hair cells were still intact. Massive cell death occurred in the middle turn thereafter and gradually spread to the basal turn, resulting in secondary degeneration of spiral ganglion neurons in the corresponding cochlear locations. These results demonstrated that Cx26 plays essential roles in postnatal maturation and homoeostasis of the organ of Corti before the onset of hearing.

Copyright information:

© 2009 Elsevier Inc. All rights reserved.

This is an Open Access work distributed under the terms of the Creative Commons Attribution-NonCommerical-NoDerivs 3.0 Unported License (http://creativecommons.org/licenses/by-nc-nd/3.0/).

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