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Super-resolution structured illumination fluorescence microscopy of the lateral wall of the cochlea: the Connexin26/30 proteins are separately expressed in man
KTH, School of Engineering Sciences (SCI), Applied Physics, Cell Physics. KTH, Centres, Science for Life Laboratory, SciLifeLab.ORCID iD: 0000-0002-5584-9170
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2016 (English)In: Cell and Tissue Research, ISSN 0302-766X, E-ISSN 1432-0878, 1-15 p.Article in journal (Refereed) PublishedText
Abstract [en]

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.

Place, publisher, year, edition, pages
Springer, 2016. 1-15 p.
Keyword [en]
Connexin (as elsewhere) 26/30, Human cochlea, Structured illumination microscopy
National Category
Biochemistry and Molecular Biology
URN: urn:nbn:se:kth:diva-187272DOI: 10.1007/s00441-016-2359-0ISI: 000378877600003ScopusID: 2-s2.0-84960093889OAI: diva2:929655

QC 20160519

Available from: 2016-05-19 Created: 2016-05-19 Last updated: 2016-07-26Bibliographically approved

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Blom, Hans
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Cell PhysicsScience for Life Laboratory, SciLifeLab
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