Representative confocal microscopy images (left) of C6-ceramide uptake and intracellular distribution and its quantification (right). The uptake was assayed on fibroblasts derived from affected individuals and control subjects. Data are means SEM (n = 9). For each independent biological replicate, a region measuring 990 x 1,320 m was imaged and analyzed, comprising approximately one hundred fibroblasts. Asterisks indicate statistical significance:*p < 0.05;**p < 0.01;***p < 0.001 for p.Ser800Leu fibroblasts vs. pool of control fibroblasts; p < 0.05; p < 0.01; for p.Asp849Glu fibroblasts vs. pool of control fibroblasts. Scale bar = 100 m.
