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Regulation of cell junctions by ClCN2 in cystic fibrosis
Author(s) -
Henry Katherine,
Lee Seakwoo,
Zeitlin Pamela
Publication year - 2016
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.30.1_supplement.1223.20
Subject(s) - cystic fibrosis , microbiology and biotechnology , secretion , tight junction , cell , mutation , chloride channel , biology , chemistry , gene , endocrinology , genetics
Cystic Fibrosis (CF) is an autosomal recessive disease, characterized by mutations in the CFTR gene, incorrect protein folding, and impaired chloride secretion. Ultimately, patients with CF exhibit a change in membrane potential regulation and it is possible that some of these changes are the result of impaired cell junctions. Previous data from our lab shows there is a targeted localization of ClCN2 to areas of cell junctions when portions of the c‐terminus are removed (ClCN2‐584). New data shows that this targeted localization is associated with a significant increase in transepithelial resistance in vitro . We hypothesize that this targeted localization and increase in TER suggests a potentially important role for ClCN2 in the regulation of tight junctions during CF, including changes in protein expression, protein interactions and tight junction function. Support or Funding Information 5T32HL72748‐11: Ruth L Kirschstein National Research Service Award