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toms Sko for salgひっちぃ2015年10月4日 To understand how the F508del mutation disrupts CFTR channel gating, several investigators have examined the gating kinetics of the F508del CFTR Cl channel. Dalemans et al14 first demonstrated that in cell attached membrane patches on Vero cells voltage clamped at 60 mV, the F508del mutation was without effect on open times, but decreased mean closed times five fold compared with those of wild type CFTR. Haws et al20 and our group23, 26 examined the gating kinetics of F508del CFTR in membrane patches from BHK and C127 cells. Both groups found that the F508del mutation was without effect on open and closed times within bursts. Instead, F508del caused a large decrease in Po by (i) markedly prolonging the closed time interval between bursts and (ii) reducing mean burst duration20, 23, 26. These data suggest that the F508del mutation disrupts CFTR channel gating in two ways: first, F508del dramatically slows the rate of entry into a burst of channel activity. Second, F508del accelerates the rate of channel closure. [参考] |
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