Table 1.
Factor/Cells/Time | GM13025/XPCS1BA | GM13026/XPCS2BA | GM21071/XP33BR | GM21072/XP183MA | GM21153/XP181MA |
---|---|---|---|---|---|
0 0.5 1 3 24h | 0 0.5 1 3 24h | 0 0.5 1 3 24h | 0 0.5 1 3 24h | 0 0.5 1 3 24h | |
XPC | 3+ 3+ 3+ 3+ 2+ | 3+ 3+ 3+ 3+ 2+ | 3+ 3+ 3+ 3+ 2+ | 3+ 3+ 3+ 3+ 2+ | 3+ 3+ 3+ 3+ 2+ |
XPB | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ | - - - - - | - - - - - |
XPD | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ |
p62 | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ | 2+ 3+ 3+ 3+ 2+ |
MAT1 | - - - - - | - - - - - | - - - - - | - - - - - | - - - - - |
XPG | nd nd nd nd nd | - 1+ 2+ 2+ - | - 1+ 2+ 2+ 1+ | - 2+ 3+ 3+ 2+ | - 2+ 3+ 3+ 2+ |
*Relative post-irradiation recruitment and dispersal kinetics of various NER and TFIIH factors at localized DNA damage sites were examined in XP-B (XP33BR), XP-B/CS fibroblasts from mild symptom patients (XPCS1BA, XPCS2BA), and in fibroblasts from severe symptom patients (XP181MA, XP183MA). Scores of -, 1+, 2+ and 3+ respectively represent < 20, 20-40, 40-60 and 60-80% of the cognate factor-positive foci in the cell population. The percentage are calculated based on foci and cell counts from more than three fields. “nd” indicates “not done”.