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. 2009 Aug 31;132(11):3165–3174. doi: 10.1093/brain/awp221

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© The Author(s) 2009.

Published by Oxford University Press on behalf of the Guarantors of Brain. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/2.5/uk/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Histochemical staining of the muscle biopsy from Patient 7 at 3 months of age (A and B) and 9 years (C and D) and of the muscle from his asymptomatic mother (E and F). The early biopsy confirmed severe mitochondrial myopathy with RRF (A) and even more COX-negative fibres (B). These changes significantly improved but did not completely disappear at 9 years of age (C and D). The muscle biopsy from the mother revealed a few COX-negative (F), SDH hyper-reactive (E) fibres (stars). A, C: Gomori trichrome; B, D, F: COX; E: SDH stain. Magnification: A, B 100×, C, D, E, F 200×.