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. 2014 Dec 10;9:183. doi: 10.1186/s13023-014-0183-8

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© Desplantes et al.; licensee BioMed Central. 2014

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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Pathological features of the muscle in patient UPN5248 with myopathic syndrome. (A) Serial skeletal muscle sections of biopsy from patient 1 showed mild variation in fiber size (HE = hematoxylin and eosin stain) and type 1 muscle fiber predominance (ATPase 9.40). With oxidative enzyme reactions (NADH, COX), the mesh of the intermyofibrillar network appeared thickened and slightly clumped, especially in type 2 fibers, which exhibit mainly glycolytic metabolism. (B) Skeletal muscle section of a biopsy from patient stained with PAS stain, showing intense coloration in all muscle fibers.