Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
8819647 | Journal of Cystic Fibrosis | 2018 | 6 Pages |
Abstract
An increased understanding of CFTR structure and function has provided opportunity for the development of a number of novel modulators targeting mutant CFTR however, it is important to also consider other ion channels and transporters present in the airways as putative targets for drug development. In this review, we discuss recent advances in CFTR biology which will contribute to further drug discovery in the field. We also examine developments to inhibit the epithelial sodium channel (ENaC) and potentially activate alternative chloride channels and transporters as a multi-tracked strategy to hydrate CF airways and restore normal mucociliary clearance mechanisms in a manner independent of CFTR mutation.
Keywords
ABCASLUTPNHENa-K-Cl cotransporterNKCC1NBDΔF508pKaCFTRcACCENaCNa+/H+ exchangersmall-interfering RNAsiRNAAdenosine TriphosphateATPUridine triphosphatenucleotide binding domaincystic fibrosis transmembrane conductance regulatorCystic fibrosisanion exchangerProteasesprotein kinase Aepithelial sodium channelIon channelHATCaPATP binding cassetteIon transporter
Related Topics
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Medicine and Dentistry
Pulmonary and Respiratory Medicine
Authors
S. Lorraine Martin, Vinciane Saint-Criq, Tzyh-Chang Hwang, László Csanády,