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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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Autofluorescent lipofusin inclusions in muscle biopsies from LOPD. (a) LOPD fiber (pt. NBSL9a) viewed in fluorescence (top) and transmitted light (bottom) shows autofluorescent inclusions directly surrounded by myofibrils. Fluorescence was excited at 488 nm and collected from 467 to 499 nm. Transmitted light is with DIC contrast. Bar: 25 μm. (b) LOPD fiber (pt. NBSL2) with prominent inclusions. The fiber was stained with LAMP2 (lysosomes: green) and LC3 (autophagosomes: red). Some inclusions are seen within the lysosome or autolysosome (arrows) whereas others appear free in the cytoplasm (asterisks). Bar: 10 μm. (c) Autofluorescent inclusions stain positive for Oil Red. The fiber (isolated from muscle biopsy of pt. D3) was also stained with LAMP2 (green). Bar: 10 μm. (d) Sudan Black B staining demonstrates lipofuscin accumulation in a fiber from pt. D3. Bar: 10 μm.