RT期刊文章SR电子T1病理粘液和粘液间隙受损的囊性纤维化患者因浓度增加而导致,未改变培养基JF欧洲呼吸杂志Jo EUR RESPIR J FD欧洲呼吸学会SP 1801297 DO 10.1183 / 13993003.01297-2018 VO 52是6 A1山188bet官网地址大卫B. A1长,罗伯特·F A1 Kissner,威廉J. A1阿提埃赫,教育暨青年局A1 Garbarine,伊恩C. A1 Markovetz,马修R. A1丰塔纳,尼古拉斯C. A1克里斯蒂,马修A1 Habibpour,迈赫迪A1 Tarran,罗伯特·A1 Forest,M. Gregory A1 Boucher,Richard C. A1按钮,Brian YR 2018 UL //www.qdcxjkg.com/content/52/6/1801297.abstract/52/6/1801297.abstract Ab囊性纤维化(CF)是一种隐性遗传疾病其特点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