Figure 6

F508del-CFTR correctors correct the trafficking of SUR1 mutants V187D and A116P. Representative immunoblots are shown for SUR1 V187D (A-C) and SUR1 A116P (D-F). Representative immunoblot for cells expressing SUR1 V187D treated with decreasing concentrations of ouabain (ouab), KM57, and RDR1 (A), KM60, ABT-888, and latonduine (latond) (B), glafenine and carbamazepine (carbam) (C). Representative immunoblot for cells expressing SUR1 A116P treated with 10% glycerol (glycerol), decreasing concentrations of ouabain and glafenine (D), carbamazepine (carbam), KM57, and KM60 (E), latonduine, RDR1, ABT-888 (F). In all blots, lanes expressing vector controls (vector), Kir6.2 in the absence of SUR1 (−), and wild type SUR1 (wt) are shown. Lanes where Kir6.2 and SUR1 V187D or SUR1 A116P are expressed are indicated by a line above the corresponding lanes. Each blot has a vehicle control (DMSO) and positive controls (glibenclamide; DMSO, 29°C). Arrows indicate core-glycosylated SUR1 protein (core), complex-glycosylated SUR1 protein (complex, indicating rescue). Tubulin is shown as a loading control. In panels A-C, an asterisk indicates a non-specific band.