Virally Mediated Connexin 26 Expression in Postnatal Scala Media Significantly and Transiently Preserves Hearing in Connexin 30 Null Mice.

Autor:	Zhang L; Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.; Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, United States.; Department of Cell Biology, Emory University School of Medicine, Atlanta, GA, United States., Wang W; Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.; Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, United States.; Department of Cell Biology, Emory University School of Medicine, Atlanta, GA, United States., Kim SM; Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, United States.; Department of Cell Biology, Emory University School of Medicine, Atlanta, GA, United States., Wang J; Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, United States.; Department of Cell Biology, Emory University School of Medicine, Atlanta, GA, United States., Zhou B; Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, United States.; Department of Cell Biology, Emory University School of Medicine, Atlanta, GA, United States., Kong W; Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China., Zheng J; Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, United States.; Department of Cell Biology, Emory University School of Medicine, Atlanta, GA, United States., Lin X; Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, United States.; Department of Cell Biology, Emory University School of Medicine, Atlanta, GA, United States.
Jazyk:	angličtina
Zdroj:	Frontiers in cell and developmental biology [Front Cell Dev Biol] 2022 Apr 27; Vol. 10, pp. 900416. Date of Electronic Publication: 2022 Apr 27 (Print Publication: 2022).
DOI:	10.3389/fcell.2022.900416
Abstrakt:	Non-sensory cells in the sensory epithelium of the cochlea are connected extensively by gap junctions. Functionally null mutations in GJB6 (encoding Cx30) cause hearing loss in humans. In this study, we injected AAV1-CB7- Gjb2 into the scala media between P0-2 in the cochlea of Gjb6 -/- mice. The injection increased Cx26 expression and significantly preserved auditory functions. However, the hearing preservation gradually declined and essentially disappeared 3 months after the injections. In contrast, the morphological preservation was still significant at 3 months post-injection. We found that the expression of Cx26, at both the mRNA and protein levels, showed substantial decreases during the 3-month period. Curiously, treatments by injecting AAV1-CB7- Gjb6 with the identical approach failed to yield any hearing preservation. Our results demonstrated the first successful cochlear gene therapy treatment in mouse models by virally expressing a companion gene of Gjb6 . Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2022 Zhang, Wang, Kim, Wang, Zhou, Kong, Zheng and Lin.)
Databáze:	MEDLINE
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