Microscopy from Scientific Research

Fibroblasts were incubated with BSA-gold (10 nm) for 24 h and washed and then the gold was chased overnight to accumulate in lysosomal compartments. Cells were then studied by transmission electron microscopy. Images of untreated fibroblasts are found on the left (Ctrl) whereas images on the right side (24 h) represent fibroblasts stained with BSA-gold. In BJ control fibroblasts, most of the gold tracer is found in uniformly dense lysosomes. In patient-derived cells, the gold is found in the dense part of bigger lysosomal compartments, which mostly consist of an electron-lucent lumen. This phenotype is similar for the various patient-derived fibroblasts. Scale bars: 1 μm.

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