| Increased Sensitivity of Porcine ΔF508-CFTR Chloride Channel to Specific Small Molecule CFTR Inhibitors |
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| 基金项目： | Supported by the General Administration of Quality Supervision,Inspection and Quarantine of China(No.2006IK145);;the Natural Science Foundation of Jilin Province,China(Nos.200705269 and 20030708);;the Natural Science Foundation of Changchun City,China(No.2007sf19);;the Science and Technology Fund from the Education Department of Liaoning Province,China(No.20060492) |
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| 摘 要： | Deletion of the codon encoding the phenylalanine residue at position 508(ΔF508) in the cystic fibrosis transmembrane conductance regulator(CFTR) is the most common mutation causing cystic fibrosis(CF).The human ΔF508 mutation results in a CFTR protein with impaired folding,trafficking,and gating in human and rodents.Recent studies suggest that pig ΔF508-CFTR can be efficiently processed to the plasma membrane as maturely glycosylated protein,indicating species difference of the molecular mechanisms of CFTR ...
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| 关 键 词： | 氯离子通道 猪组织 敏感性 抑制剂 小分子 基因突变 分子机制 功能特性 |
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