Egyszerű nézet Martin SL Saint-Criq V Hwang TC Csanády, László 2018-10-11T06:54:54Z 2018-10-11T06:54:54Z 2018
dc.identifier 85033447749
dc.identifier.citation pagination=S22-S27; journalVolume=17; journalIssueNumber=2; journalTitle=JOURNAL OF CYSTIC FIBROSIS;
dc.identifier.uri doi:10.1016/j.jcf.2017.10.006
dc.description.abstract Lung health relies on effective mucociliary clearance and innate immune defence mechanisms. In cystic fibrosis (CF), an imbalance in ion transport due to an absence of chloride ion secretion, caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) and a concomitant sodium hyperabsorption, caused by dyregulation of the epithelial sodium channel (ENaC), results in mucus stasis which predisposes the lungs to cycles of chronic infection and inflammation leading to lung function decline. 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.
dc.relation.ispartof urn:issn:1569-1993
dc.title Ion channels as targets to treat cystic fibrosis lung disease
dc.type Journal Article 2018-09-28T06:35:44Z
dc.language.rfc3066 en
dc.identifier.mtmt 3287704
dc.identifier.wos WOS:000440265700005
dc.identifier.pubmed 29102290
dc.contributor.department SE/AOK/I/Orvosi Biokémiai Intézet
dc.contributor.institution Semmelweis Egyetem
dc.mtmt.swordnote FELTÖLTŐ: Bökönyi Zita -

Kapcsolódó fájlok:

A fájl jelenleg csak egyetemi IP címről érhető el.


Ez a rekord az alábbi gyűjteményekben szerepel:

Egyszerű nézet