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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
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Otolaryngology and Head and Neck Surgery.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology. Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Otolaryngology and Head and Neck Surgery.
Royal Inst Technol, Dept Appl Phys, Sci Life Lab, Tomtebodavagen 23A, S-17121 Solna, Sweden..
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Immunology, Genetics and Pathology, Clinical Immunology.
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2016 (English)In: Cell and Tissue Research, ISSN 0302-766X, E-ISSN 1432-0878, Vol. 365, no 1, 13-27 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
2016. Vol. 365, no 1, 13-27 p.
Keyword [en]
Human cochlea, Connexin (as elsewhere) 26/30, Structured illumination microscopy
National Category
Cell and Molecular Biology
Identifiers
URN: urn:nbn:se:uu:diva-299834DOI: 10.1007/s00441-016-2359-0ISI: 000378877600003PubMedID: 26941236OAI: oai:DiVA.org:uu-299834DiVA: diva2:950292
Available from: 2016-07-29 Created: 2016-07-28 Last updated: 2016-07-29Bibliographically approved

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Liu, WeiEdin, FredrikMagnusson, PeetraLi, HaoLaurell, GöranRask-Andersen, Helge
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Otolaryngology and Head and Neck SurgeryClinical Immunology
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