BAAV mediated GJB2 gene transfer restores gap junction coupling in cochlear organotypic cultures from deaf Cx26Sox10Cre mice

The deafness locus DFNB1 contains GJB2, the gene encoding connexin26 and GJB6, encoding connexin30, which appear to be coordinately regulated in the inner ear. In this work, we investigated the expression and function of connexin26 and connexin30 from postnatal day 5 to adult age in double transgeni...

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Veröffentlicht in:	PloS one 2011-08, Vol.6 (8), p.e23279-e23279
Hauptverfasser:	Crispino, Giulia, Di Pasquale, Giovanni, Scimemi, Pietro, Rodriguez, Laura, Galindo Ramirez, Fabian, De Siati, Romolo Daniele, Santarelli, Rosa Maria, Arslan, Edoardo, Bortolozzi, Mario, Chiorini, John A, Mammano, Fabio
Format:	Artikel
Sprache:	eng
Schlagworte:	Animal models Animals Biology Cattle Cochlea Cochlea - pathology Cochlea - physiopathology Communication Connexin 26 Connexins Connexins - genetics Connexins - therapeutic use Coupling Deafness Deafness - pathology Deafness - physiopathology Deafness - therapy Dependovirus - metabolism Electrophysiological Phenomena Fluorescence Recovery After Photobleaching Gap Junctions - metabolism Gene therapy Gene transfer Genes Genetic engineering Genetic Therapy Genetic vectors GJB2 protein Hair Cells, Auditory - metabolism Hair Cells, Auditory - pathology Health aspects Hearing loss Inner ear Integrases - metabolism Medicine Mice Morphogenesis Mutation Organ Culture Techniques Organ of Corti - pathology Permeability Physiology Recombinant Fusion Proteins Recovery (Medical) Restoration Sox10 protein SOXE Transcription Factors - metabolism Therapeutic applications Time Factors Transduction, Genetic Transgenic mice Viruses
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