Research Paper Volume 5, Issue 6 pp 445—459

Progeroid laminopathy with restrictive dermopathy-like features caused by an isodisomic LMNA mutation p.R435C

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Figure 7.

53BP1 localization in cultured human normal fibroblasts transfected with either mCherry-tagged LMNA-p.R435C or mCherry-tagged wt- LMNA plasmids. (A) untreated or (B) treated with hydrogen peroxide (H2O2). 53BP1 was specifically labeled using a monoclonal antibody and revealed by FITC –conjugated anti-mouse IgG secondary antibody (green). (C) Quantitation of 53BP1 foci in H2O2 –treated cells. Means of three different counts performed in separate experiments are reported +/− standard deviation. The difference is statistically significant (p<0.05 by the Student's T-test).