A stabilizing influence: CAL PDZ inhibition extends the half-life of Δf508-CFTR

Abstract

It pays to recycle! The most common mutation in cystic fibrosis (CF) impedes maturation and accelerates breakdown of the ion channel CFTR. A "stabilizer" has been characterized that blocks a PDZ domain responsible for CFTR degradation. The inhibitor iCAL36 extends the chloride channel's half-life in airway epithelial cells. It also complements the activity of a corrector of the maturation defect, thus suggesting the potential for combination CF therapies. Copyright © 2010 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.