نتایج جستجو برای: connexin cx26

تعداد نتایج: 5395  

Journal: :American journal of physiology. Lung cellular and molecular physiology 2001
Y Guo C Martinez-Williams C E Yellowley H J Donahue D E Rannels

Extracellular matrix (ECM) proteins promote attachment, spreading, and differentiation of cultured alveolar type II epithelial cells. The present studies address the hypothesis that the ECM also regulates expression and function of gap junction proteins, connexins, in this cell population. Expression of cellular fibronectin and connexin (Cx) 43 increase in parallel during early type II cell cul...

2017
Liang Xu Andrea Carrer Francesco Zonta Zhihu Qu Peixiang Ma Sheng Li Federico Ceriani Damiano Buratto Giulia Crispino Veronica Zorzi Gaia Ziraldo Francesca Bruno Chiara Nardin Chiara Peres Flavia Mazzarda Anna M. Salvatore Marcello Raspa Ferdinando Scavizzi Youjun Chu Sichun Xie Xuemei Yang Jun Liao Xiao Liu Wei Wang Shanshan Wang Guang Yang Richard A. Lerner Fabio Mammano

Background: Mutations leading to changes in properties, regulation, or expression of connexin-made channels have been implicated in 28 distinct human hereditary diseases. Eight of these result from variants of connexin 26 (Cx26), a protein critically involved in cell-cell signaling in the inner ear and skin. Lack of non-toxic drugs with defined mechanisms of action poses a serious obstacle to t...

Journal: :Archives of otolaryngology--head & neck surgery 2001
S Marlin E N Garabédian G Roger L Moatti N Matha P Lewin C Petit F Denoyelle

OBJECTIVE To evaluate difficulties encountered in genetic counseling in deaf children carrying connexin 26 gene (CX26 or GJB2) mutations. DESIGN Prospective study. SETTING Outpatients, tertiary referral center. PATIENTS Ninety-six unrelated deaf children in whom CX26 mutations had been detected consecutively. Children were recruited to a center for genetic counseling for deaf children, an...

2015
Mariana C. Fiori Srinivasan Krishnan D. Marien Cortes Mauricio A. Retamal Luis Reuss Guillermo A. Altenberg Luis G. Cuello

Gap-junction channels (GJCs) communicate the cytoplasm of adjacent cells and are formed by head-to-head association of two hemichannels (HCs), one from each of the neighbouring cells. GJCs mediate electrical and chemical communication between cells, whereas undocked HCs participate in paracrine signalling because of their permeability to molecules such as ATP. Sustained opening of HCs under pat...

Journal: :The Journal of neuroscience : the official journal of the Society for Neuroscience 2013
Anne-Cécile Boulay Francisco J del Castillo Fabrice Giraudet Ghislaine Hamard Christian Giaume Christine Petit Paul Avan Martine Cohen-Salmon

Gjb2 and Gjb6, two contiguous genes respectively encoding the gap junction protein connexin26 (Cx26) and connexin 30 (Cx30) display overlapping expression in the inner ear. Both have been linked to the most frequent monogenic hearing impairment, the recessive isolated deafness DFNB1. Although there is robust evidence for the direct involvement of Cx26 in cochlear functions, the contribution of ...

2017
Eric Press Katanya C Alaga Kevin Barr Qing Shao Felicitas Bosen Klaus Willecke Dale W Laird

Several mutant mice have been generated to model connexin (Cx)-linked skin diseases; however, the role of connexins in skin maintenance and during wound healing remains to be fully elucidated. Here we generated a novel, viable, and fertile mouse (Cx26CK14-S17F/+) with the keratitis-ichthyosis-deafness mutant (Cx26S17F) driven by the cytokeratin 14 promoter. This mutant mouse mirrors several Cx2...

Journal: :Histology and histopathology 2007
M J Azanza N Pes R N Pérez-Bruzón J Aisa M Raso C Junquera J M Lahoz C Maestú C Martínez-Ciriano C Pérez-Castejón A Vera-Gil A Del Moral

The aim of the present study was to examine the distribution of cells expressing connexin 26 (Cx26) in the suboesophageal visceral, left and right parietal and left and right pleural ganglia of the snail Helix aspersa by immunocytochemistry. Altogether we have found approximately 452 immunoreactive neurons which represent the 4.7% of the total neurons counted. The stained large neurons (measure...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1996
J X Jiang D A Goodenough

Gap junction channels are formed by paired oligomeric membrane hemichannels called connexons, which are composed of proteins of the connexin family. Experiments with transfected cell lines and paired Xenopus oocytes have demonstrated that heterotypic intercellular channels which are formed by two connexons, each composed of a different connexin, can selectively occur. Studies by Stauffer [Stauf...

Journal: :iranian journal of public health 0
m hashemzadeh chaleshtori l hoghooghi rad m dolati r sasanfar a hoseinipour m montazer zohour

while hearing loss has been considered to be a very heterogeneous disorder, mutations in gap junction beta 2 (gjb2) gene encoding connexin 26 (cx26) protein are the major cause of autosomal recessive and sporadic non-syndromic deafness in many populations. in this study, we have investigated the prevalence of the gjb2 gene mutations using nested pcr pre screening strategy and direct sequencing ...

Journal: :Frontiers in bioscience 2016
Yunfeng Wang Chen Lin Yingzi He Ao Li Wenli Ni Shan Sun Xiaodong Gu Jian Li Huawei Li

To investigate the underlying molecular mechanism for connexin 26 (Cx26) knockout-induced apoptosis, we performed TUNEL assays to detect apoptosis in the cochlear sensory epithelium in Cx26 knockout mice. We also compared the miRNA expression profiles of Cx26 knockout and wild-type mice using microarray technology and bioinformatic analyses. Real-time PCR, luciferase reporter gene assays, and s...

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