Protecting SMN-Deficient Cells from Oxidative Stress

We study how cells lacking SMN respond to oxidative stress, which makes them more vulnerable to DNA damage and reduces their growth. The main problem is repairing oxidative DNA lesions, as key repair proteins cannot bind properly in SMN-deficient cells.
We investigate these repair processes in living cells and test whether antioxidants, such as N-acetyl-L-cysteine (NAC), can improve survival. We also examine how nerve injury and muscle denervation disrupt nucleoli in muscle cells and collaborate with SMA mouse model studies to better understand disease mechanisms.
This research aims to identify strategies to protect SMA cells and inform potential therapies.