CFTR, a plasma membrane anion channel, plays a key role in controlling transepithelial fluid movement. Excessive activation results in intestinal fluid loss during secretory diarrhoeas, while CFTR mutations underlie cystic fibrosis (CF). Anion permeability depends both on how well CFTR channels work (permeation/gating) and on how many are present at the membrane (reflecting folding, trafficking, metabolic stability). Recently, treatments with two drug classes targeting CFTR – one boosting ion-channel function (potentiators), the other increasing plasma membrane density (correctors) – have provided significant health benefits to CF patients.

中文翻译：

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同时定量CFTR离子通道功能和质膜接近度的荧光检测

CFTR是质膜阴离子通道，在控制跨上皮液运动中起关键作用。过度激活会导致分泌性腹泻期间肠液流失，而CFTR突变是囊性纤维化（CF）的基础。阴离子渗透率既取决于CFTR通道的工作性能（渗透/门控），又取决于膜上存在的通道数量（反映折叠，运输，代谢稳定性）。最近，使用针对CFTR的两种药物进行治疗-一种增强离子通道功能（增强剂），另一种增强质膜密度（校正剂）-为CF患者带来了显着的健康益处。