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. 2019 Nov 27;9:17640. doi: 10.1038/s41598-019-54158-2

Figure 1.

Figure 1

Co-potentiator ASP-11 activity in minimal function CFTR mutants in transfected FRT cells. (A) Structure of ASP-11. (B) Location of minimal function CFTR mutants studied herein. (C) Representative short-circuit current data in FRT cells expressing I1234del-CFTR, Q1313X-CFTR and S492F-CFTR in response to 20 µM forskolin (fsk), 5 µM VX-770, 20 µM ASP-11 and 10 μM CFTRinh-172. As indicated, cells were treated with 3 µM VX-809 for 18–24 h prior to measurements. (n = 3) (D). Representative short-circuit current data in FRT cells expressing predicted W1282X-CFTR read-through products W1282L-CFTR, W1282R-CFTR and W1282C-CFTR (without and with VX-809 pretreatment) (n = 3). MSD, membrane spanning domain; NBD, nucleotide binding domain; R, regulatory domain.