TY - JOUR T1 -钠通道阻滞剂和三磷酸尿苷:effect on nasal potential difference in cystic fibrosis mice(囊性纤维化小鼠的鼻部电势差异)N2 -钠通道抑制剂阻断囊性纤维化(CF)中增强的Na+再吸收。胞外核苷酸通过Ca2+门控Cl通道促进Cl-分泌。综上所述,CF的粘稠性分泌物较少,更容易咳痰。本研究检测了钠通道阻断剂与三磷酸尿苷(UTP)联合应用对CF插入空突变小鼠(cftr(tm1HGU))、deltaF508纯合小鼠(cftr(tm1Cam))和配对对照动物的鼻膜电位差(PD)的影响。插入型CF小鼠和deltaF508型CF小鼠基础PD中位数分别为-28 mV和-34 mV。这些数值与对照组动物有显著差异(-20 mV)。阿米洛利和洛哌丁胺降低cftr(tm1HGU) CF小鼠PD (deltaPD 13 mV &15 mV分别)表示Na+封锁。 The subsequent addition of UTP in a chloride-free vehicle increased the PD (deltaPD -8- -12.5 mV). DeltaF508 mice showed significantly greater responses compared with CF insertional null mutant mice (p<0.05). The action of UTP was brief and not prolonged by the addition alpha-beta-methylene-adenosine 5' diphosphate. Suramin, a competitive antagonist of P2 purinoceptors blocked the action of UTP. In conclusion, this study demonstrated dose dependant nasal membrane potential changes in differences mice with uridine triphosphate in the presence of sodium channel blockers suggestive of chloride secretion. More stable analogues of uridine triphosphate in combination with long acting sodium channel blockers such as loperamide may have therapeutic potential in cystic fibrosis. ER -