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Such defects need to be treated with separate pharmacological compounds termed potentiators and correctors, respectively. Here, we report the characterization of aminoarylthiazoles AATs as compounds having dual activity. Cells expressing mutant CFTR were studied with functional assays fluorescence-based halide transport and short circuit current measurements to assess the effect of acute and chronic treatment with compounds. We found that AATs are effective on Fdel, the most frequent cystic fibrosis mutation, which is associated with both a gating and a trafficking defect. Incubation of cells expressing mutant CFTR with ENI caused a strong stimulation of channel activity as demonstrated by single channel recordings. Compounds with dual activity such as AATs may be useful for the development of effective drugs for the treatment of cystic fibrosis. CF is caused by mutations in the cystic fibrosis transmembrane conductance regulator CFTR gene, which codes for a plasma membrane protein expressed at the apical surface of epithelial cells in the airways, intestine, pancreas, and other organs.
Economic literature: papers , articles , software , chapters , books. Citations Many of the citations below have been collected in an experimental project, CitEc , where a more detailed citation analysis can be found. These are citations from works listed in RePEc that could be analyzed mechanically. So far, only a minority of all works could be analyzed. See under "Corrections" how you can help improve the citation analysis. Blog mentions As found by EconAcademics. Baumol, William J.
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William J. Baumol
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