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. 2016 Feb 18;7(2):e2105. doi: 10.1038/cddis.2015.374

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Copyright © 2016 Macmillan Publishers Limited

Cell Death and Disease is an open-access journal published by Nature Publishing Group. This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article's Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

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Pharmacological evaluation of the 11 prelamin A modulators on HGPS defects. (a) Measure of nuclear shape abnormalities (lamin A/C immunostaining) in HGPS MSCs following 48 h of treatment with each of the 11 prelamin A modulators. Each chart represents the mean±S.D. of 8 independent experiments. (b) Lamin A/C immunostaining of WT and HGPS MSCs following 48 h of treatments with each of the three Mono-APs (Mono-AP1, Mono-AP2 and Mono-AP3). Scale bar represents 50 μm. (c) Measure of osteogenic differentiation (Alkaline phosphatase activity) in HGPS MSCs following 7 days of differentiation in presence of each of the 11 prelamin A modulators. Each chart represents the mean±S.D. of eight independent experiments. Data are normalized on cell number. (d) Alkaline phosphatase activity of WT and HGPS MSCs following 7 days of differentiation in presence of each of the three Mono-APs (Mono-AP1, Mono-AP2 and Mono-AP3). Scale bar represents 50 μm. (e) Measure of cell proliferation (Ki-67 immunostaining) in HGPS MSCs following 48 h of treatments with each of the 11 prelamin A modulators. Each chart represents the mean±S.D. of eight independent experiments. (f) Measure of percent of confluence in long-term culture in presence of Mono-APs (Mono-AP1, Mono-AP2 and Mono-AP3)