Bypassing CFTR dysfunction in cystic fibrosis with alternative pathways for anion transport

• Verfasst von: Li, Hongyu [VerfasserIn]
• Verfasst von: Salomon, Johanna J. [VerfasserIn]
• Verfasst von: Mall, Marcus A. [VerfasserIn]
• Titel: Bypassing CFTR dysfunction in cystic fibrosis with alternative pathways for anion transport
• Verf.angabe: Hongyu Li, Johanna J Salomon, David N Sheppard, Marcus A Mall and Luis JV Galietta
• E-Jahr: 2017
• Jahr: 21th October 2017
• Umfang: 7 S.
• Fussnoten: Gesehen am 12.09.2018
• Titel Quelle: Enthalten in: Current opinion in pharmacology
• Ort Quelle: Amsterdam [u.a.] : Elsevier Science, 2001
• Jahr Quelle: 2017
• Band/Heft Quelle: 34(2017), Seite 91-97
• ISSN Quelle: 1471-4973
• DOI: doi:10.1016/j.coph.2017.10.002
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1580917615

Abstract

One therapeutic strategy for cystic fibrosis (CF) seeks to restore anion transport to affected epithelia by targeting other apical membrane Cl− channels to bypass dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl− channel. The properties and regulation of the Ca2+-activated Cl− channel TMEM16A argue that long-acting small molecules which target directly TMEM16A are required to overcome CFTR loss. Through genetic studies of lung diseases, SLC26A9, a member of the solute carrier 26 family of anion transporters, has emerged as a promising target to bypass CFTR dysfunction. An alternative strategy to circumvent CFTR dysfunction is to deliver to CF epithelia artificial anion transporters that shuttle Cl− across the apical membrane. Recently, powerful, non-toxic, biologically-active artificial anion transporters have emerged.