Fig. 3.

Immunofluorescent studies in fibroblasts.

Cultured skin fibroblasts from patient 1 (A–C) and an age-matched healthy control (D–F). Mitotracker in red shows reduced staining in the patient (A). Complex IV immunostaining in green shows reduction and heterogeneity in the patient's cells (B). Panel C shows segregation of staining: the presence of complex IV positive mitochondria (green) that lack mitochondrial membrane potential sensitive Mitotracker staining. Cell nuclei were stained with DAPI (blue in all panels). Panels D to F show normal staining in a control cell line. Scale bars = 50 μm.