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. 2016 Feb 5;4:9. doi: 10.1186/s40478-016-0276-9

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© Sandell et al. 2016

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. 4 — a, b, c, d, e. The same patient and muscle as in Fig. 3. The rimmed vacuoles showed material with reactivity for several markers of defect degradation and autophagic processing such as ubiquitin (a), VCP (b), TDP-43 (c), p62 (d) and SMI-31 (e). f, g. The rimmed vacuolar regions of local degeneration filled with autophagosomes, shown by LC3 (f) reactivity, components of mature lysosomes expressing LAMP2 (g)