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. 2018 Dec 13;9:1464. doi: 10.3389/fphar.2018.01464

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Copyright © 2018 Pesce, Sondo, Ferrera, Tomati, Caci, Scudieri, Musante, Renda, Baatallah, Servel, Hinzpeter, di Bernardo, Pedemonte and Galietta.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Effect of USP13 or USP10 overexpression. (A) Functional analysis in CFBE41o- transiently transfected with plasmids expressing USP13/USP10 or with a control plasmid. Cells also stably expressed F508del-CFTR and the HS-YFP. After transfection, cells were treated with VX-809 (1 μM) or with vehicle. The figure shows representative traces (top) and summary of QR for the different conditions (n = 4 independent experiments; ^∗p < 0.05; ^∗∗∗p < 0.001). (B) Effect of spautin-1 on cells transfected with USP13 and treated with/without VX-809. Data obtained with the HS-YFP assay. Spautin-1 markedly abolishes F508del-CFTR function in USP13-transfected cells (n = 4–7 independent experiments; ^∗p < 0.05; ##/^∗∗p < 0.01; ###/^∗∗∗p < 0.001).