Figure 2.

Differential effects of the small molecules CFFT‐004 and C18 on the maturation of F508del‐ and A561E‐CFTR expressed in BHK cells. (A) Representative Western blots of BHK cells stably expressing F508del‐ and A561E‐CFTR are shown. CF mutant‐expressing BHK cells were treated with CFFT‐004 (5 μM), C18 (5 μM) or the vehicle DMSO (0.05% v·v⁻¹) for 24 h at 37°C before cells were lysed and Western blotting performed; other CF mutant‐expressing BHK cells were untreated (ut). Lysates from BHK cells stably expressing wild‐type CFTR were used as a control. CFTR was detected with the mouse anti‐CFTR monoclonal antibody (596), which recognizes a region of NBD2 (1204–1211; Cui et al., 2007). Arrows indicate the positions of the band B (immature) and C (mature) forms of CFTR. (B) The amount of CFTR protein present in the mature form (band C) is expressed as a percentage of total CFTR protein [% CFTR protein processed = (band C / [bands B + C] × 100)]. Data are means ± SEM (n = 13) expressed as a percentage of that of wild‐type CFTR.