Additional file 10: of HR23B pathology preferentially co-localizes with p62, pTDP-43 and poly-GA in C9ORF72-linked frontotemporal dementia and amyotrophic lateral sclerosis

Figure S7. Nucleotide excision repair is not affected in C9ORF72 human fibroblasts. A) Dose-response curve for 4 healthy control human fibroblast lines (81E253, 86E1375, 06E0717 and 99E0774) and 4 C9ORF72 human fibroblast lines (13E634, 13E659, 17E0225, 17E0278) and the NER deficient XP25RO human fibroblast line treated with increasing dose of UV-C light (0–12 J/m2). B) Immunofluorescence staining showing the recruitment of NER factors XPC, XPB, XPA, XPF and XPG to local DNA damage (visualized by CPD antibody), induced by 60 J/m2 UV-C irradiation through a microporous filter. Fibroblast lines used for pictures: 13E634, 13E659 and 81E253 C) Human fibroblasts lines were treated with 16 J/m2 UV-C light and incubated with EdU for 1 h to measure unscheduled DNA synthesis (UDS) as measure of DNA repair. The NER-deficient XPC25RO cell line is shown as negative control. Ctrl 1–4 are lines 81E253, 86E1375, 06E0717 and 99E0774 in this order. C9 1–4 are lines 13E634, 13E659, 17E0225, 17E0278 in this order. (PDF 934 kb)