Results 41 to 50 of about 4,249 (178)
GJB2 and GJB6 are adjacent genes encoding connexin 26 (Cx26) and connexin 30 (Cx30), respectively, with overlapping expressions in the inner ear. Both genes are associated with the commonest monogenic hearing disorder, recessive isolated deafness DFNB1 ...
Junmin Chen +28 more
doaj +1 more source
Divalent regulation and intersubunit interactions of human Connexin26 (Cx26) hemichannels [PDF]
Control of plasma membrane connexin hemichannel opening is indispensable, and is achieved by physiological extracellular divalent ion concentrations. Here, we explore the differences between regulation by Ca(2+) and Mg(2+) of human connexin26 (hCx26) hemichannels and the role of a specific interaction in regulation by Ca (2+).
Lopez, William +3 more
openaire +14 more sources
Mutations in gap junction beta-2 (GJB2), the gene that encodes connexin 26 (CX26), are the most frequent cause of hereditary deafness worldwide. We recently developed an in vitro model of GJB2-related deafness (induced CX26 gap junction-forming cells ...
Ichiro Fukunaga +7 more
doaj +1 more source
ISCs generate spontaneous activity in the absence of Cx26.
(a) (Top) Schematic depicting cross section of the immature cochlea with targeted recording site for electrophysiology and calcium imaging from ISCs indicated.
Calvin J. Kersbergen (16455643) +3 more
core +1 more source
Mutations of Cx26 gene (GJB2) for prelingual deafness in Taiwan [PDF]
Mutations in the Cx26 (GJB2) gene have been shown to be responsible for a major part of autosomal recessive non-syndromic inherited prelingual deafness. We have sequenced the coding region of GJB2 gene from 169 Taiwanese patients with prelingual deafness and 100 unrelated normal individuals.
Yi-Chun, Wang +7 more
openaire +2 more sources
Alterations of gap junctional intercellular communication appear to play a role in the development and progression of cancer. Gap junction channel is composed of two connexons - hexameric units formed of transmembrane proteins called connexins (Cxs). The
Stanislaw Sulkowski +6 more
doaj +1 more source
Sensorineural hearing loss and the incidence of Cx26 mutations in Austria [PDF]
A clinical evaluation and Cx26 mutation analysis was performed in 92 consecutive patients with sensorineural hearing loss in order to delineate the spectrum of genetically caused hearing loss. Among patients of Austrian origin, 53% were classified with hereditary hearing loss. Cx26 mutations were found in 26% of NSHL patients (40% of familial vs 18% of
J, Löffler +6 more
openaire +2 more sources
Mutations in the GJB2 gene encoding transmembrane protein connexin 26 (Cx26) are the most common cause for hearing loss worldwide. Cx26 plays a crucial role in the ionic and metabolic homeostasis in the inner ear, indispensable for normal hearing process.
Ekaterina A. Maslova +2 more
doaj +1 more source
Investigation of Ion Permeation through the Cx26 Hemichannel [PDF]
Connexins form the intercellular channels composing gap junctions in vertebrates and providing both the electronic coupling and the exchange of ions and small molecules between adjacent cells. A gap junction channel consists of two opposed hemichannels that were recently shown to be functional when embedded in a nonjunctional membrane.
Kasimova, Marina +3 more
openaire +1 more source
Delayed Cx30 plaque formation in Cx26 cKO inner supporting cells.
(a) Immunostaining for Connexin 26 (green) in whole mount middle P7 cochlea from control (Gjb2fl/fl) and Cx26 cKO (Tecta-Cre;Gjb2fl/fl) mice. Hair cells (magenta) are labeled by immunoreactivity to Myosin VIIA.
Calvin J. Kersbergen (16455643) +3 more
core +1 more source

