Progeroid laminopathy with restrictive dermopathy-like features caused by an isodisomic LMNA mutation p.R435C
Figure 6.
Staining with anti-gamma H2AX (phospho S139) antibody of samples taken from skin (a), esophagus (b) and skeletal muscle (c) during autopsy at age of 11 months shows a strong signal, indicating presence of DNA double strand breaks.