%0期刊文章%一个山,大卫B.%A Long,Robert F.%A Kissner,William J.%A Atieh,Eyad%Garbarine,Ian C.%A Markovetz,Matthew R.%A Fontana,Nicholas C。%A Christy,Matthew%A Habibpour,Mehdi%A Tarran,Robert%A Forest,M. Gregory%A Boucher,Richard C.%A按钮,Brian%T病理粘液和粘液纤维化患者的粘液间隙受损患者浓度,未改变pH%D 2018%R 10.1183 / 13993003.01297-2018%J.01297-2018%J欧洲呼吸轴颈%p 1801297%v 52%n 6%n 6%x囊性纤维化(cf)是一种隐性遗传疾病,其特征是气道粘液堵塞和减少粘液间隙。目前有目前的替代假设,试图描述CF Airways病的标志性的异常粘性和弹性粘液,包括:1)CF跨膜调节剂(CFTR) - 依赖性气道表面积(水)分泌,产生粘液超浓度 -依赖性增加的粘度和2)CFTR的碳酸氢盐分泌受损,产生气道表面的酸化和增加的粘液粘度。进行一系列实验,以确定粘液浓度与pH的贡献,以跨长度鳞片的气道粘液的流变特性。nanoscopic to macroscopic.For length scales greater than the nanoscopic, i.e. those relevant to mucociliary clearance, the effect of mucus concentration dominated over the effect of airway acidification.Mucus hydration and chemical reduction of disulfide bonds that connect mucin monomers are more promising therapeutic approaches than alkalisation.Over length scales greater than the mesh size of the mucin polymeric network of mucus, concentration, and not pH, dominates the physical properties of mucus that are associated with cystic fibrosis airways disease http://ow.ly/K92u30m2RDC %U //www.qdcxjkg.com/content/erj/52/6/1801297.full.pdf