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Connexins in the Inner Ear

  • Chapter
Connexins

Abstract

Intercellular communication via gap junctions is crucial for auditory function. This has been emphasized by the findings that mutations in certain connexin genes, in particular GJB2 and GJB6 (encoding CX26 and CX30), cause sensorineural deafness. Cx26 and Cx30 proteins are widely expressed in the epithelial and connective tissues of the cochlea and vestibular system, where they likely form heteromeric gap junction channels. Despite the study of mutant channels and of mouse models for both recessive and dominant autosomal deafness, it is still unclear why gap junctions are essential for auditory function and why Cx26 and Cx30 cannot compensate for loss of each other. It is generally thought that gap junctions play a role in the maintenance of ionic and metabolic homeostasis in the inner ear. Recent studies highlight the possible involvement of gap junctions in intercellular signaling via second messengers between the nonsensory cells. This chapter summarizes current knowledge about the molecular and functional properties of inner ear gap junctions and the inner ear pathologies associated with connexin mutations.

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Acknowledgments

The authors are supported by the Biotechnology and Biological Sciences Research Council (BBSRC), Deafness Research UK, The Royal National Institute for Deaf People (RNID), and the Royal Society. We apologize to colleagues whose work could not be cited owing to space restrictions.

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  1. Centre for Auditory Research, UCL Ear Institute, University College London, 332 Gray’s Inn Road, London, WC1X 8EE, UK

    Andrew Forge

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  1. Regina Nickel
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Nickel, R., Forge, A., Jagger, D. (2009). Connexins in the Inner Ear. In: Harris, A.L., Locke, D. (eds) Connexins. Humana Press. https://doi.org/10.1007/978-1-59745-489-6_20

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