Gianluca Cestra

This study investigates the distinct toxic effects of QAGR and PACL peptides in Drosophila, showing they impair nucleolar function and autophagy or alter stress granule dynamics, contributing to DM2 pathogenesis.

This study shows that mutant FUS reduces ALKBH5 expression, increasing m6A levels, and that restoring m6A alleviates ALS pathology in Drosophila models.

This study shows that TDP-43 and FUS cytoplasmic inclusions impair DNA damage response and repair, and that pharmacological stimulation of DDR can rescue these defects in ALS models.

This study shows that FUS-induced splicing changes lead to cytoplasmic accumulation of hnRNP A2b/B1 splice variants, promoting stress granule formation and motor neuron toxicity in a mouse model of ALS.