Section Reviews: Pulmonary-Allergy, Dermatological, Gastrointestinal & Arthnlis: Pharmacological approaches to correct the bioelectric deficits in cystic fibrosis

Abstract

Despite recent progress in determining the genetic basis of cystic fibrosis (CF), treatment of this disease is almost entirely based on antibiotic therapy to treat lung infections arising from viscous mucous and impaired mucociliary clearance. No treatments are presently available that address the underlying defect created by mutation of the cystic fibrosis transmembrane regulator (CFTR) protein. However, there is currently a great deal of interest in correcting this defect by gene therapy (ineffective at present) and by correcting the erroneous processing of CFTR present with some mutations (early research). In addition, pharmacological treatments aimed at correcting the underlying defect produced by the absence of the CFTR chloride channel are currently under investigation. Pharmacological treatments aimed at the epithelial sodium channel and the alternative chloride channel are the focus of current research reviewed in this article.