Fig. 6

Aberrant Sec31A morphology in ALS-C9orf72 patient fibroblasts. (A) Confocal images following immunocytochemistry of Sec31A vesicle clusters in control (n = 2) or ALS-C9orf72 patient (n = 1) derived fibroblasts. (B) Quantification of the number of Sec31A-positive clusters, paired t-test, *p < 0.05. (C) Quantification of fold-change in area of each Sec31A positive vesicular cluster in C9orf72 fibroblasts relative to average vesicle area of control fibroblasts. ****p < 0.0001. (D) Quantification of fold-change in intensity of Sec31A staining in each cluster in C9orf72 fibroblasts relative to average staining intensity of control fibroblasts. ****p < 0.0001. (E) Quantification of change in circularity of detected clusters. Circularity was obtained from ImageJ (ranging from 0, – not circular, to 1, – fully circular) and the difference to average circularity of control fibroblasts was calculated. ****p < 0.0001. Mean ± SEM of individual clusters depicted. (B)-(E) data points represent technical replicates using two different antibodies to Sec31A