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. 2014 Sep 16;2:137. doi: 10.1186/s40478-014-0137-3

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© Hiniker et al.; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. 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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Paraspinal muscle pathology. Histochemical and ultrastructural analysis of T8 paraspinal muscle demonstrated evidence of a mitochondrial myopathy. (a) H&E stain (arrows indicate fibers with increase in basophilic staining indicative of mitochondrial hyperplasia), (b) modified trichrome stain (arrows indicate ragged red fibers), (c) COX (cytochrome c oxidase) stain (asterisks mark COX-negative fibers). Electron microscopy (d-f) shows mitochondrial hyperplasia, variation in the mitochondrial shape and size, and mitochondria with crystalline arrays (arrowheads). Of note, the crystalline arrays present in this case do not have a common “parking lot” appearance. Scale bars: a-c, 50 µm; d, 1.5 µm; e, 1 µm; f, 200 nm.