Figure 6.
Effect of VX-445-based treatments on the conformational stability of mutant CFTR. (A) Immunoblot detection of CFTR in whole lysates derived from wild-type or F508del-CFTR expressing CFBE41o- cells treated with vehicle alone (DMSO), or (for mutant CFTR only) with VX-445 (3 µM), VX-809 (3 µM), or VX-661 (10 µM) as single agents or combinations, following heat-denaturation at 28–70 °C. For comparison, whole lysates derived from parental CFBE41o- cells (null cells) are also shown as controls for antibody specificity. (B) Quantification of aggregation-resistant (soluble) CFTR band C by densitometry, normalized by HSP90AB1 expression (means ± SD; n = 3).