Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA; The Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
Department of Cell Developmental and Integrative Biology, University of Alabama at Birmingham, Birmingham, AL, USA; The Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, AL, USA.
Curr Opin Pharmacol. 2017 Jun;34:132-139. doi: 10.1016/j.coph.2017.09.013. Epub 2017 Nov 10.
Airways diseases, especially chronic obstructive pulmonary disease (COPD) and asthma, are common causes of morbidity and mortality worldwide. There is an ongoing unmet need for novel and effective therapies. There is an established pathophysiological link and phenotypic similarity between the chronic bronchitis phenotype of COPD and cystic fibrosis (CF). New evidence suggests that CFTR dysfunction may play a role in other common airways diseases such as COPD, non-atopic asthma and non-CF bronchiectasis. Newly approved and investigational drugs that target both mutant and wild-type CFTR channels have provided a new treatment opportunity addressing the mucus defect in pulmonary diseases that share the same pathophysiology with CF.
气道疾病,尤其是慢性阻塞性肺疾病(COPD)和哮喘,是全球发病率和死亡率的常见原因。目前仍需要新型有效的治疗方法。COPD 的慢性支气管炎表型和囊性纤维化(CF)之间存在既定的病理生理学联系和表型相似性。新的证据表明,CFTR 功能障碍可能在其他常见气道疾病中起作用,如 COPD、非特应性哮喘和非 CF 支气管扩张症。新批准和正在研究的靶向突变和野生型 CFTR 通道的药物为具有相同病理生理学的肺部疾病的黏液缺陷提供了新的治疗机会。