Super-resolution structured illumination fluorescence microscopy of the lateral wall of the cochlea: the Connexin26/30 proteins are separately expressed in man

Wei Liu, Fredrik Edin, Hans Blom, Peetra Magnusson, Annelies Schrott-Fischer, Rudolf Glueckert, Peter A. Santi, Hao Li, Göran Laurell, Helge Rask-Andersen

Research output: Contribution to journalArticlepeer-review

21 Scopus citations

Abstract

Globally 360 million people have disabling hearing loss and, of these, 32 million are children. Human hearing relies on 15,000 hair cells that transduce mechanical vibrations to electrical signals in the auditory nerve. The process is powered by the endo-cochlear potential, which is produced by a vascularized epithelium that actively transports ions in conjunction with a gap junction (GJ) system. This “battery” is located “off-site” in the lateral wall of the cochlea. The GJ syncytium contains the GJ protein genes beta 2 (GJB2/connexin26 (Cx26)) and 6 (GJB6/connexin30 (Cx30)), which are commonly involved in hereditary deafness. Because the molecular arrangement of these proteins is obscure, we analyze GJ protein expression (Cx26/30) in human cochleae by using super-resolution structured illumination microscopy. At this resolution, the Cx26 and Cx30 proteins were visible as separate plaques, rather than being co-localized in heterotypic channels, as previously suggested. The Cx26 and Cx30 proteins thus seem not to be co-expressed but to form closely associated assemblies of GJ plaques. These results could assist in the development of strategies to treat genetic hearing loss in the future.

Original languageEnglish (US)
Pages (from-to)13-27
Number of pages15
JournalCell and Tissue Research
Volume365
Issue number1
DOIs
StatePublished - Jul 1 2016

Bibliographical note

Funding Information:
This study was supported by ALF grants from Uppsala University Hospital and Uppsala University and by the Foundation of “Tysta Skolan,” the Swedish Deafness Foundation (HRF) and generous private donations from Börje Runögård and David Giertz, Sweden. This work was also funded in part by Medel (Fuerstenweg 77a, 6020 Innsbruck, Austria). Funding was also provided by NIDCD (PS - U24DC011968). Imaging was performed with equipment maintained by the Science for Life Lab BioVis Platform, Uppsala University. For skillful art work we thank Karin Lodin.

Funding Information:
This study was supported by ALF grants from Uppsala University Hospital and Uppsala University and by the Foundation of "Tysta Skolan," the Swedish Deafness Foundation (HRF) and generous private donations from B??rje Run??g??rd and David Giertz, Sweden. This work was also funded in part by Medel (Fuerstenweg 77a, 6020 Innsbruck, Austria). Funding was also provided by NIDCD (PS - U24DC011968). Imaging was performed with equipment maintained by the Science for Life Lab BioVis Platform, Uppsala University. For skillful art work we thank Karin Lodin.

Publisher Copyright:
© 2016, Springer-Verlag Berlin Heidelberg.

Copyright:
Copyright 2019 Elsevier B.V., All rights reserved.

Keywords

  • Connexin (as elsewhere) 26/30
  • Human cochlea
  • Structured illumination microscopy

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