Figure 2

Figure 2

Decreased mitochondria, typical cristae changes and increased fusion activity of mitochondrial dynamics in the myopathy under electronic microscopy. (A) In longitudinal sections of the specimen of patient 5, mitochondria decreased obviously, and distributed fairly sparsely in some areas of some muscle fibres (electron microscope (EM), 20 000×). (B) Abnormality of the cristae of mitochondria in patient 4, including lack of definition (black arrow), simplification of the cristae (white arrow), parallel cristae (white arrow), homogenised matrix (black arrowhead) and circular cristae (white arrowhead). (C) In patient 5, many elongated mitochondria can be observed in several myofibers, some of which are branched (arrow) (EM, 20 000×); (D) in patient 5, an elongated mitochondria with normal diameter (EM, 80 000×).