Figure 4.
Muscle biopsy in patients with mitochondrial disease. a | In a patient with MERRF (harbouring a mitochondrial DNA point mutation that impairs mitochondrial protein synthesis), two ragged-red fibres are evident with the modified Gomori trichrome stain. b–d | Using the SDH stain, the same fibres appear ‘ragged blue’ (part b) but are COX-negative (part c), and stain blue with the combined COX and SDH stain (COX-deficient fibres stain a lighter blue; part d). Abbreviations: MERRF, myoclonus epilepsy with ragged-red fibres; SDH, succinate dehydrogenase. Image first printed in Molecular Neurology.155