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. 2014 Jan 2;2:2. doi: 10.1186/2051-5960-2-2

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Copyright © 2014 Feeney et al.; licensee BioMed Central Ltd.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. 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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Autophagic abnormalities and autofluorescent inclusions in adult-onset patients. a-c: Muscle fibers were stained for lysosomal marker LAMP2 (green) and autophagosomal marker LC3 (red). (a) LOPD fiber (pt. D16) shows mild lysosomal enlargement and clusters of autophagosomes. (b) Autophagic buildup and autofluorescent inclusions represent a major pathology in this fiber (pt. D7). (c) Autofluorescent inclusions are seen within the LAMP2-positive structures (pt. D7). Bar in a-c: 10 μm. (d) Both autophagic buildup and inclusions are missed by routine histology; the image shows epon-embedded PAS-stained section of muscle biopsy from pt. D15 (10x).