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. 2018 Aug 28;8:28. doi: 10.1186/s13395-018-0173-y

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© The Author(s). 2018

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided 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 Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Fig. 3 — Expression of p.P104L missense mutant CAV3 disturbs ER-Golgi integrity. (a) EM findings in p.P104L CAV3 mutant quadriceps muscle: subsarcolemmal accumulation of vesicular structures (most likely corresponding to abnormal caveolae) (black arrows in 3A.1 and 3A.2) and dispersed ERGIC-Golgi structures (black arrows in 3A.3 – 3A.5). (b) Immunohistochemical studies focusing on ER-Golgi stress confirm GM130 increase. (c) Immunofluorescence studies focusing on Golgi-structure in 6 month and 1 year old animals (visualization via golgin-97 staining) revealed a statistically significant dispersion on p.P104L CAV3 transgenic animals