Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis

Ann Am Thorac Soc. 2016 Apr;13 Suppl 2(Suppl 2):S169-76. doi: 10.1513/AnnalsATS.201509-601KV.

Abstract

Chronic obstructive pulmonary disease is a common cause of morbidity and a rising cause of mortality worldwide. Its rising impact indicates the ongoing unmet need for novel and effective therapies. Previous work has established a pathophysiological link between the chronic bronchitis phenotype of chronic obstructive pulmonary disease and cystic fibrosis as well as phenotypic similarities between these two airways diseases. An extensive body of evidence has established that cigarette smoke and its constituents contribute to acquired dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein in the airways, pointing to a mechanistic link with smoking-related and chronic bronchitis. Recent interest surrounding new drugs that target both mutant and wild-type CFTR channels has paved the way for a new treatment opportunity addressing the mucus defect in chronic bronchitis. We review the clinical and pathologic evidence for modulating CFTR to address acquired CFTR dysfunction and pragmatic issues surrounding clinical trials as well as a discussion of other ion channels that may represent alternative therapeutic targets.

Keywords: chronic bronchitis; chronic obstructive pulmonary disease; cystic fibrosis transmembrane conductance regulator.

Publication types

  • Review
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Anti-Bacterial Agents / therapeutic use*
  • Bronchitis, Chronic / metabolism
  • Bronchitis, Chronic / therapy*
  • Cystic Fibrosis / metabolism
  • Cystic Fibrosis / therapy
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
  • Expectorants / therapeutic use*
  • Humans
  • Physical Therapy Modalities*
  • Smoking / metabolism

Substances

  • Adrenal Cortex Hormones
  • Anti-Bacterial Agents
  • CFTR protein, human
  • Expectorants
  • Cystic Fibrosis Transmembrane Conductance Regulator