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. 2022 Sep 15;132(18):e154571. doi: 10.1172/JCI154571

Figure 3. The eRF3a degrader CC-90009 rescues W1282X-CFTR in a panel of cell lines and parent primary cells.

Figure 3

(A–C) TECC-24 measurements of the UNCX2T cell line (W1282X/W1282X) treated with 0.1% DMSO, 200 μM G418, 0.3 μM SMG1i, 0.1 μM CC-90009, and 3 μM VX-809, alone or in combination as indicated. Acute addition of the potentiator 10 μM VX-770 is indicated by an arrow. (A) TECC-24 tracing representing 3–4 replicates. (B and C) ΔIeq in response to FSK (B) and CFTRinh-172 (C). Data were analyzed using ordinary linear models. n = 3–4. (D–I) TECC-24 measurements of a panel of W1282X/W1282X cell lines and parent cells treated with 0.1% DMSO or 0.1 μM CC-90009. (D and E) TECC-24 tracing of the UNCX2T cell line (D) and parent cells (E). Tracings are representative of the W1282X/W1282X panel containing 3 cell donors with 6 replicates per donor. (F–I) ΔIeq in response to FSK (F) and CFTRinh-172 (G), benazmil (H), and basal Ieq (I). Data were analyzed using a linear mixed-effects model with the donor as a random effect factor. n = 6 per donor. Post hoc comparisons were performed using the general linear hypothesis test. All data are presented as the mean ± SD. ***P < 0.001.