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. 2018 Jan 17;8:977. doi: 10.1038/s41598-017-16667-w

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© The Author(s) 2018

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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Expression of recombinant C1-INH. (a) Evaluation of secreted and intracellular rC1-INH in COS-7 measured by ELISA. Transfected COS-7 cell cultures were investigated for C1-INH concentration in supernatants (full bars) and in lysates (dotted bars), for non-transfected (control) and transfected cells (rWT and r378C for wild-type and Arg378Cys minigene plasmid, respectively). The activation in the presence of 1,000 U/mL interferon-γ is indicated (IFN). P values were calculated from the unpaired Student’s t tests. (b) Evaluation of secreted rC1-INH in Hepa 1.6 measured by Dot Blot. Transfected Hepa 1.6 cell cultures were investigated for C1-INH concentration in supernatants with and without IFN-γ for wild-type (open bars) and Arg378Cys (closed bars). On the left a representative Dot Blot is shown.