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• W2914692893 endingPage "L86" @default.
• W2914692893 startingPage "L71" @default.
• W2914692893 abstract "Cross-species comparative studies have highlighted differences between human and mouse cystic fibrosis transmembrane conductance regulator (CFTR), the epithelial Cl − channel defective in cystic fibrosis (CF). Here, we compare the impact of the most common CF mutation F508del on the function of human and mouse CFTR heterologously expressed in mammalian cells and their response to CFTR modulators using the iodide efflux and patch-clamp techniques. Once delivered to the plasma membrane, human F508del-CFTR exhibited a severe gating defect characterized by infrequent channel openings and was thermally unstable, deactivating within minutes at 37°C. By contrast, the F508del mutation was without effect on the gating pattern of mouse CFTR, and channel activity demonstrated thermostability at 37°C. Strikingly, at all concentrations tested, the clinically approved CFTR potentiator ivacaftor was without effect on the mouse F508del-CFTR Cl − channel. Moreover, eight CFTR potentiators, including ivacaftor, failed to generate CFTR-mediated iodide efflux from CHO cells expressing mouse F508del-CFTR. However, they all produced CFTR-mediated iodide efflux with human F508del-CFTR-expressing CHO cells, while fifteen CFTR correctors rescued the plasma membrane expression of both human and mouse F508del-CFTR. Interestingly, the CFTR potentiator genistein enhanced CFTR-mediated iodide efflux from CHO cells expressing either human or mouse F508del-CFTR, whereas it only potentiated human F508del-CFTR Cl − channels in cell-free membrane patches, suggesting that its action on mouse F508del-CFTR is indirect. Thus, the F508del mutation has distinct effects on human and mouse CFTR Cl − channels." @default.
• W2914692893 created "2019-02-21" @default.
• W2914692893 creator A5019426685 @default.
• W2914692893 creator A5021790168 @default.
• W2914692893 creator A5023055657 @default.
• W2914692893 creator A5023506885 @default.
• W2914692893 creator A5043109979 @default.
• W2914692893 creator A5045663774 @default.
• W2914692893 creator A5057117256 @default.
• W2914692893 creator A5088996192 @default.
• W2914692893 date "2019-07-01" @default.
• W2914692893 modified "2023-10-17" @default.
• W2914692893 title "Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR" @default.
• W2914692893 cites W1482359363 @default.
• W2914692893 cites W1503660683 @default.
• W2914692893 cites W1516227220 @default.
• W2914692893 cites W1601195737 @default.
• W2914692893 cites W1606430679 @default.
• W2914692893 cites W1677528798 @default.
• W2914692893 cites W1805119337 @default.
• W2914692893 cites W1903125214 @default.
• W2914692893 cites W1966648102 @default.
• W2914692893 cites W1975971473 @default.
• W2914692893 cites W1976507289 @default.
• W2914692893 cites W1977373496 @default.
• W2914692893 cites W1984195830 @default.
• W2914692893 cites W1991013623 @default.
• W2914692893 cites W1991977085 @default.
• W2914692893 cites W1995692151 @default.
• W2914692893 cites W1998770772 @default.
• W2914692893 cites W2004844038 @default.
• W2914692893 cites W2009065727 @default.
• W2914692893 cites W2012138378 @default.
• W2914692893 cites W2012280093 @default.
• W2914692893 cites W2013914743 @default.
• W2914692893 cites W2015881103 @default.
• W2914692893 cites W2032157023 @default.
• W2914692893 cites W2035131077 @default.
• W2914692893 cites W2039599894 @default.
• W2914692893 cites W2043338020 @default.
• W2914692893 cites W2049914201 @default.
• W2914692893 cites W2050657251 @default.
• W2914692893 cites W2051015480 @default.
• W2914692893 cites W2054767423 @default.
• W2914692893 cites W2054965620 @default.
• W2914692893 cites W2066722784 @default.
• W2914692893 cites W2067891202 @default.
• W2914692893 cites W2069109845 @default.
• W2914692893 cites W2071164110 @default.
• W2914692893 cites W2071407356 @default.
• W2914692893 cites W2073266239 @default.
• W2914692893 cites W2075257287 @default.
• W2914692893 cites W2078078890 @default.
• W2914692893 cites W2078818437 @default.
• W2914692893 cites W2079234445 @default.
• W2914692893 cites W2080083110 @default.
• W2914692893 cites W2080281443 @default.
• W2914692893 cites W2080476066 @default.
• W2914692893 cites W2082102563 @default.
• W2914692893 cites W2084896237 @default.
• W2914692893 cites W2086293424 @default.
• W2914692893 cites W2088118742 @default.
• W2914692893 cites W2096580943 @default.
• W2914692893 cites W2096914283 @default.
• W2914692893 cites W2099916828 @default.
• W2914692893 cites W2103543801 @default.
• W2914692893 cites W2107339692 @default.
• W2914692893 cites W2110652155 @default.
• W2914692893 cites W2110892460 @default.
• W2914692893 cites W2112032807 @default.
• W2914692893 cites W2112048001 @default.
• W2914692893 cites W2112146921 @default.
• W2914692893 cites W2113165479 @default.
• W2914692893 cites W2116321050 @default.
• W2914692893 cites W2116885714 @default.
• W2914692893 cites W2124233675 @default.
• W2914692893 cites W2125460059 @default.
• W2914692893 cites W2125639616 @default.
• W2914692893 cites W2133873320 @default.
• W2914692893 cites W2135063244 @default.
• W2914692893 cites W2136017423 @default.
• W2914692893 cites W2136656278 @default.
• W2914692893 cites W2141023256 @default.
• W2914692893 cites W2146826437 @default.
• W2914692893 cites W2151837778 @default.
• W2914692893 cites W2158288696 @default.
• W2914692893 cites W2160821931 @default.
• W2914692893 cites W2162989130 @default.
• W2914692893 cites W2166215338 @default.
• W2914692893 cites W2167724548 @default.
• W2914692893 cites W2233070782 @default.
• W2914692893 cites W2256298007 @default.
• W2914692893 cites W2270680140 @default.
• W2914692893 cites W2414828124 @default.
• W2914692893 cites W2528194947 @default.
• W2914692893 cites W2579431806 @default.
• W2914692893 cites W2583650046 @default.
• W2914692893 cites W2611404350 @default.

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