Lipid droplets are dynamic organelles with key functions in lipid and energy homeostasis. There are several factors involved in lipid droplets metabolism, such as Fatty-Acid Transport Protein (fatp). In recent years, it has been reported that lipid droplets play a role in neuron survival and could response to different stresses such as lipotoxicity, oxidative stress and unpaired protein homeostasis. Dysregulation of the unfolded protein response (UPR) can lead neurodegenerative diseases and the generation of lipid droplets. The contribution of the UPR pathways, Ire1/Xbp1 and ire1/RIDD, in lipid droplet formation is not known.
In my project, I studied whether the ire1/RIDD pathway targets fatp and in turn affects the generation of lipid droplets in both physiological and pathological conditions, ninaEG69D and ninaAE110V mutants, which are associated with endoplasmic reticulum stress activation. Besides, I investigated the lipid storage dysregulation in another pathological condition named β-propeller associated neurodegeneration (BPAN).