期刊
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
卷 100, 期 4, 页码 1937-1942出版社
NATL ACAD SCIENCES
DOI: 10.1073/pnas.2627982100
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资金
- NIDDK NIH HHS [DK54759, DK25295, P30 DK054759] Funding Source: Medline
To better understand the function of the conserved C terminus of the cystic fibrosis (CF) transmembrane conductance regulator, we studied constructs containing deletions in the C-terminal tail. When expressed in well differentiated CF airway epithelia, each construct localized predominantly to the apical membrane and generated transepithelial Cl- current. The results suggested that neither the C-terminal PSD-95/Discs-large/ZO-1 (PDZ)-interacting motif nor other C-terminal sequences were absolutely required for apical expression in airway epithelia. Surprisingly, deleting an acidic cluster near the C terminus reduced both channel opening rate and transepithelial Cl- transport, indicating that it influences channel gating. These results may help explain the relative paucity of CF-associated mutations in the C terminus.
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