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. 2018 Dec 17;9:5342. doi: 10.1038/s41467-018-07718-5

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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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Fig. 1 — Cytoplasmic protein aggregation by BAG3P^209L. a Schematic representation of BAG3 depicting the WW domain, the IPV motifs, the PxxP domain and the BAG domain. The disease-causing mutations P209L, P209Q, and P209S, and p470S are indicated with an arrow. The Hsp70-interaction disrupting mutation R480A is indicated with an *. b Immunofluorescence pictures of myoblast expressing FLAG-BAG3^WT or FLAG-BAG3^P209L, using BAG3 (green) or FLAG (red) antibodies. DAPI staining is shown in blue. Scale bar = 5 μm. c Immunofluorescence pictures of HeLa cells expressing FLAG-BAG3^P209L using antibodies against BAG3 (green) and Lamin A/C (red). DAPI staining is shown in blue. Scale bar = 5 μm. d Immunofluorescence pictures of FLAG-BAG3^WT and FLAG-BAG3^P209L expressing HeLa cells using BAG3 antibody (green) before and after detergent treatment prior to fixation. Scale bar = 5 μm. e Whole cell extracts (WCE) and NP-40 soluble and insoluble fractions of HEK293 cells expressing indicated FLAG-BAG3 variants. Western blot against the indicated antibodies is shown. Source data are provided as a Source data file