Fig. 7

Aberrant Sec31A morphology in ALS-C9orf72 patient spinal cord tissues. (A) Confocal images following immunohistochemistry for Sec31A of control or ALS-C9orf72 patient spinal cords (n = 3 of each). Last panel shows staining after threshold setup in Fiji Image J. Scale bar: 20 µm. Star: lipofuscin area was excluded from the region analysed. Dashed area: region of interest for Sec31A analysis. Neurons were identified by their characteristic size and morphology. Arrows: larger Sec31A puncta were observed in control patients compared to ALS-C9orf72 patient motor neurons. (B) Quantification of the average Sec31A puncta size. At least six neurons from each control and ALS-C9orf72 patient were analysed. Mean ± SEM from three control and three patient tissues; student’s t test, ** p < 0.01. Sec31A puncta were significantly smaller in ALS-C9orf72 patient neurons compared to control cells. (C) Quantification of Sec31A puncta count/area. Mean ± SEM from three control and three patients; student’s t test, * p < 0.05. ALS-C9orf72 neurons displayed significantly more Sec31A puncta per area compared to control patient cells. (D) Quantification of the percentage area of total Sec31A puncta between controls and patient motor neurons, ns = non significant