Figure 2.
Localization of mutant LRPPRC to mitochondria in LSFC fibroblasts. Fibroblasts from control and one LSFC patient were grown on coverslips and incubated with antibodies against LRPPRC and against cytochrome c followed by incubation with secondary antibodies coupled to red and green fluorescent dyes, respectively. The overlay of these two images demonstrates the mitochondrial localization of mutant LRPPRC in patient fibroblasts and confirms the reduction in mutant LRPPRC levels in LSFC fibroblasts.