Fig 2: XPC and TFIIH are continuously recruited to UV damage in XPCS patient fibroblasts. (A) Representative immunofluorescence pictures showing the co-localization of XPF and XPB at LUD in patient fibroblasts XPCS1CD and CS1USAU, compared to wild type C5RO fibroblasts. Cells were stained 1 h and 8 h after local UV irradiation (60 J/m2) through an 8 μm microporous filter with antibodies against XPF and XPB. (B) Representative immunofluorescence pictures showing the LUD recruitment of XPD and XPC in fibroblasts derived from patients carrying mutations in XPB, XPD, XPG, XPF and XPA, affected by mild XP (XP42RO, XP32BR, XP6BE), XPCS (XPCS1CD, XPCS1BA, XPCS2, XPCS1RO) or DSC (XP25RO) compared to wild type C5RO fibroblasts. Cells were locally UV irradiated with 60 J/m2 UVC through an 8 μm microporous filter and stained 1 h and 8 h after irradiation with antibodies against XPD and XPC. Scale bar: 5 μm.

Fig 3: The NER machinery is continuously recruited at sites of DNA damage in XPF KO and XPF-C236R expressing cells. Representative immunofluorescence pictures and quantification of LUD recruitment of XPC (A and B), XPB (C and D), XPG (E and F) and XPA (G and H) 1 h and 8 h after 60 J/m2 UVC irradiation through an 8 μm microporous filter in XPF KO cells and cells expressing XPF-wt, XPF-P379S, XPF-C236R, XPF-D715A and (only in C) XPF-R589W. UV damage was visualized by CPD staining. Scale bar: 5 μm. Fold accumulation was calculated by normalizing fluorescence at sites of local damage to the nuclear background and plotted as average of at least 111 (XPC), 85 (XPB), 137 (XPG) and 105 (XPA) cells per condition from at least two independent experiments. Statistical significant difference (P < 0.05) compared to wt for each time point is indicated by *.