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• W1049405896 abstract "Connexins are components of gap junctions which facilitate transfer of small molecules between cells. One member of the connexin family, Connexin 26 (Cx26), is prevalent in gap junctions in sensory epithelia of the inner ear. Mutations of GJB2, the gene encoding Cx26, cause significant hearing loss in humans. The vestibular system, however, does not usually show significant functional deficits in humans with this mutation. Mouse models for loss of Cx26 function demonstrate hearing loss and cochlear pathology but the extent of vestibular dysfunction and organ pathology are less well characterized. To understand the vestibular effects of Cx26 mutations, we evaluated vestibular function and histology of the vestibular sensory epithelia in a conditional knockout (CKO) mouse with Cx26 loss of function. Transgenic C57BL/6 mice, in which cre-Sox10 drives excision of the Cx26 gene from non-sensory cells flanking the sensory epithelium of the inner ear (Gjb2-CKO), were compared to age-matched wild types. Animals were sacrificed at ages between 4 and 40 weeks and their cochlear and vestibular sensory organs harvested for histological examination. Cx26 immunoreactivity was prominent in the peripheral vestibular system and the cochlea of wild type mice, but absent in the Gjb2-CKO specimens. The hair cell population in the cochleae of the Gjb2-CKO mice was severely depleted but in the vestibular organs it was intact, despite absence of Cx26 expression. The vestibular organs appeared normal at the latest time point examined, 40 weeks. To determine whether compensation by another connexin explains survival of the normal vestibular sensory epithelium, we evaluated the presence of Cx30 in the Gjb2-CKO mouse. We found that Cx30 labeling was normal in the cochlea, but it was decreased or absent in the vestibular system. The vestibular phenotype of the mutants was not different from wild-types as determined by time on the rotarod, head stability tests and physiological responses to vestibular stimulation. Thus presence of Cx30 in the cochlea does not compensate for Cx26 loss, and the absence of both connexins from vestibular sensory epithelia is no more injurious than the absence of one of them. Further studies to uncover the physiological foundation for this difference between the cochlea and the vestibular organs may help in designing treatments for GJB2 mutations." @default.
• W1049405896 created "2016-06-24" @default.
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• W1049405896 date "2015-10-01" @default.
• W1049405896 modified "2023-09-24" @default.
• W1049405896 title "Mice with conditional deletion of Cx26 exhibit no vestibular phenotype despite secondary loss of Cx30 in the vestibular end organs" @default.
• W1049405896 cites W1847597758 @default.
• W1049405896 cites W1857434908 @default.
• W1049405896 cites W192047470 @default.
• W1049405896 cites W1966708427 @default.
• W1049405896 cites W1967717499 @default.
• W1049405896 cites W1969140718 @default.
• W1049405896 cites W1970118869 @default.
• W1049405896 cites W1973926811 @default.
• W1049405896 cites W1975011941 @default.
• W1049405896 cites W1975178727 @default.
• W1049405896 cites W1979208815 @default.
• W1049405896 cites W1982511534 @default.
• W1049405896 cites W1982885964 @default.
• W1049405896 cites W1984890858 @default.
• W1049405896 cites W1986530165 @default.
• W1049405896 cites W1989127634 @default.
• W1049405896 cites W1991147662 @default.
• W1049405896 cites W1998249204 @default.
• W1049405896 cites W2004339565 @default.
• W1049405896 cites W2008981305 @default.
• W1049405896 cites W2009257155 @default.
• W1049405896 cites W2011210106 @default.
• W1049405896 cites W2016502180 @default.
• W1049405896 cites W2018111258 @default.
• W1049405896 cites W2019304102 @default.
• W1049405896 cites W2019978084 @default.
• W1049405896 cites W2020476264 @default.
• W1049405896 cites W2021088854 @default.
• W1049405896 cites W2023224872 @default.
• W1049405896 cites W2023782716 @default.
• W1049405896 cites W2025147658 @default.
• W1049405896 cites W2027268676 @default.
• W1049405896 cites W2027614529 @default.
• W1049405896 cites W2028578717 @default.
• W1049405896 cites W2030167054 @default.
• W1049405896 cites W2030552324 @default.
• W1049405896 cites W2031568469 @default.
• W1049405896 cites W2033347933 @default.
• W1049405896 cites W2034060287 @default.
• W1049405896 cites W2035105931 @default.
• W1049405896 cites W2050089098 @default.
• W1049405896 cites W2050385103 @default.
• W1049405896 cites W2056943958 @default.
• W1049405896 cites W2058412782 @default.
• W1049405896 cites W2058495454 @default.
• W1049405896 cites W2063412675 @default.
• W1049405896 cites W2064581235 @default.
• W1049405896 cites W2071014369 @default.
• W1049405896 cites W2072521264 @default.
• W1049405896 cites W2077823986 @default.
• W1049405896 cites W2082040849 @default.
• W1049405896 cites W2082607874 @default.
• W1049405896 cites W2090114961 @default.
• W1049405896 cites W2092227731 @default.
• W1049405896 cites W2093241362 @default.
• W1049405896 cites W2093772720 @default.
• W1049405896 cites W2095590160 @default.
• W1049405896 cites W2099041486 @default.
• W1049405896 cites W2103113803 @default.
• W1049405896 cites W2105799240 @default.
• W1049405896 cites W2123050929 @default.
• W1049405896 cites W2123908729 @default.
• W1049405896 cites W2139237496 @default.
• W1049405896 cites W2142067945 @default.
• W1049405896 cites W2146213237 @default.
• W1049405896 cites W2146961403 @default.
• W1049405896 cites W2157162039 @default.
• W1049405896 cites W2188177736 @default.
• W1049405896 cites W22307356 @default.
• W1049405896 cites W2326411026 @default.
• W1049405896 cites W2327797159 @default.
• W1049405896 cites W77703357 @default.
• W1049405896 doi "https://doi.org/10.1016/j.heares.2015.07.018" @default.
• W1049405896 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/4581988" @default.
• W1049405896 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/26232528" @default.
• W1049405896 hasPublicationYear "2015" @default.
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