Chemical inhibition of HSF1 improves F508del-CFTR function.

Abstract

<p>Immunoblot and quantification of CFTR following treatment of F508del-CFTR–expressing CFBE cells with increasing concentration of triptolide (Trip.) alone (A) or in combination with the CF corrector VX809 (D). Results are expressed as percentage of maximum signal of CFTR band-B (set at 100%), and shown as mean ± SEM, <i>n</i>≥3; * represents <i>p</i><0.05 relative to DMSO. (C) Immunoblot and quantitative analysis of CFTR following a daily chronic dosing regimen (96 h) of 12 nM triptolide in F508del-CFTR expressing cells. Results are expressed as fold change relative to DMSO, and shown as mean ± SD, <i>n</i> = 2; * represents <i>p</i><0.05 relative to DMSO. YFP-quenching curves of F508del-CFTR expressing CFBE-YFP cells treated with the indicated compounds for 24 h alone (B) or in combination (E) (mean ± SD, <i>n</i>≥3). All results were repeated at least once. The underlying data used to make (A–E) in this figure can be found in the supplementary file <a href="http://www.plosbiology.org/article/info:doi/10.1371/journal.pbio.1001998#pbio.1001998.s008" target="_blank">Data S1</a>.</p