Georg Krainer and Antoine Treff et al. use a helical-hairpin construct derived from the cystic fibrosis transmembrane conductance regulator (CFTR) to investigate misfolding caused by the disease-linked V232D mutation. Using single-molecule FRET, they show that the V232D hairpin assumes an open conformation in lipid bilayers, which is reversed by the pharmacological corrector Lumacaftor.
- Georg Krainer
- Antoine Treff
- Michael Schlierf