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. 2015 Oct 15;5:15037. doi: 10.1038/srep15037

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Copyright © 2015, Macmillan Publishers Limited

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(A) COX/SDH histochemistry (top) and quadruple immunofluorescence (bottom, COX-I - green, NDUFB8 - purple, porin - red and laminin - white) were performed on serial muscle sections from patients P5, P9, P12 and P19. (B) Visual classification (COX/SDH) and objective classification (COX-I and porin immunodetection) results. Fibres were classified as COX (activity/protein abundance) positive (beige), intermediate(+) (light beige), intermediate(−) (light blue) or deficient (blue). Fibres counted (n = visual/immunodetection): P5 (n = 1103/841); P9: (n = 1395/1071); P12: (n = 1887/1740) and P19: (n = 956/769). Visually classified COX deficiency was overestimated in P5 by 3.6 percentage points, underestimated in P19 by 4.4 percentage points, underestimated in P12 by 4.1 percentage points, and consistent in P5, as compared to objective classification. Scale bars measure 50μm.