慢性支气管炎及其他黏液清除疾病中获得性囊性纤维化跨膜传导调节因子功能障碍
Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis and Other Diseases of Mucus Clearance.
作者信息
Raju S Vamsee, Solomon George M, Dransfield Mark T, Rowe Steven M
机构信息
Department of Medicine, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA; Department of Cell Developmental and Integrative Biology, The Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
Department of Medicine, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
出版信息
Clin Chest Med. 2016 Mar;37(1):147-58. doi: 10.1016/j.ccm.2015.11.003. Epub 2015 Dec 24.
Chronic obstructive pulmonary disease (COPD) is a major public health problem. No therapies alter the natural history of the disease. Chronic bronchitis is perhaps the most clinically troublesome phenotype. Emerging data strongly suggest that cigarette smoke and its components can lead to acquired cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction. Findings in vitro, in animal models, and in smokers with and without COPD also show acquired CFTR dysfunction, which is associated with chronic bronchitis. This abnormality is also present in extrapulmonary organs, suggesting that CFTR dysfunction may contribute to smoking-related systemic diseases.
慢性阻塞性肺疾病（COPD）是一个重大的公共卫生问题。尚无治疗方法能改变该疾病的自然病程。慢性支气管炎可能是临床上最棘手的表型。新出现的数据有力地表明，香烟烟雾及其成分可导致获得性囊性纤维化跨膜传导调节因子（CFTR）功能障碍。体外研究、动物模型以及患有和未患有COPD的吸烟者的研究结果也显示存在获得性CFTR功能障碍，这与慢性支气管炎有关。这种异常也存在于肺外器官，提示CFTR功能障碍可能导致与吸烟相关的全身性疾病。
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