Mucus-targeting therapies of defective mucus clearance for cystic fibrosis: A short review.
Mucus-targeting therapies of defective mucus clearance for cystic fibrosis: A short review.
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DOI：
10.1016/j.coph.2022.102248
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发表时间：
2022-08
影响因子：
4
通讯作者：
中科院分区：
文献类型：
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在肺中，与囊性纤维化相关的CFTR（CF）代表了气道异常粘液间隙的Nidus，因此肺部疾病有缺陷的CFTR介导的CL介导的CL介导的粘液介导的粘液特性，包括浓度，外观弹性和两种MUC5的比例。美国直接或间接的缺陷已经开发出来；然而，该评论仍然需要更好的治疗方法。
In the lungs, defective CFTR associated with cystic fibrosis (CF) represents the nidus for abnormal mucus clearance in the airways and consequently a progressive lung disease. Defective CFTR-mediated Cl− secretion results in altered mucus properties, including concentration, viscoelasticity, and the ratio of the two mucins, MUC5B and MUC5AC. In the past decades, therapies targeting the CF mucus defect, directly or indirectly, have been developed; nevertheless, better treatments to prevent the disease progression are still needed. This review summarizes the existing knowledge on the defective mucus in CF disease and highlights it as a barrier to the development of future inhaled genetic therapies. The use of new mucus-targeting treatments is also discussed, focusing on their potential role to halt the progress of CF lung disease.
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DOI：
10.1126/science.1223012
发表时间：
2012-08-24
期刊：
Science (New York, N.Y.)
影响因子：
--
作者：
Button B;Cai LH;Ehre C;Kesimer M;Hill DB;Sheehan JK;Boucher RC;Rubinstein M
通讯作者：
Rubinstein M
影响因子：
15.3
作者：
Campbell, Laura;Hepworth, Matthew R.;Grencis, Richard K.
通讯作者：
Grencis, Richard K.
影响因子：
10
作者：
Costello, CM;OConnor, CM;Hayes, JP
通讯作者：
Hayes, JP
影响因子：
24.3
作者：
Bilton, D.;Robinson, P.;Charlton, B.
通讯作者：
Charlton, B.
DOI：
10.1164/rccm.201307-1268oc
发表时间：
2013-12-15
影响因子：
24.7
作者：
Adam, Ryan J.;Michalski, Andrew S.;Stoltz, David A.
通讯作者：
Stoltz, David A.