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. 2019 Sep 3;9:12706. doi: 10.1038/s41598-019-48971-y

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© The Author(s) 2019

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Characterisation of HBH-CFTR-3HA expression and function. (a) Immunoblot analysis of CFTR-3HA and HBH-CFTR-3HA CFTR expression in CFBE. Complex- and core-glycosylated CFTR are indicated by an empty and filled arrowhead, respectively. Na/K ATPase was probed as a loading control. (b) PM densities of CFTR-3HA and HBH-CFTR-3HA were measured in CFBE with cell surface ELISA assay ECL detection. Data are means ± SEM, n = 3. (c) The CFTR-3HA- and HBH-CFTR-3HA-mediated I_sc in the presence of 20 μM forskolin. (d) The summary figure of forskolin dose-response in WT-3HA and HBH-CFTR-3HA expressed in CFBE. Data are means ± SEM. n = 3 (e) Representative I_sc recordings in WT-3HA- or HBH-CFTR-3HA- expressing CFBE. Arrows represent sequential addition of 5, 10, 30, 50, and 100 μg/ml CSC followed by addition of 20 μM Inh₁₇₂. (f) Summary of (e) Dose response curve of CSC on Inh₁₇₂-sensitive I_sc in CFBE. Data represent means ± SEM, n = 3.