Fig. 6. Abnormalities in mitochondrial ultrastructure and function in ALS patient-derived fibroblasts. (A) Transmission electron micrographs of cross-sectioned control and patient fibroblasts. N indicates the nucleus, and arrowheads indicate mitochondria. Note that patient fibroblasts show enlarged mitochondria without cristae structures. Scale bar, 0.2 µm. (B) Fluorescent images of JC-1-loaded control and patient (E1357K) fibroblasts. Note abnormal accumulation of green-fluorescent JC-1 monomers in the mitochondria of patient but not control cells. Scale bar, 15 µm.
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