Friday, 11th September 2015 - h 14:00
Seminars Room, NICO
The protein quality control system in motoneuron diseases
Dipartimento di Scienze Farmacologiche e Biomolecolari Università di Milano
Motor neuron diseases, like spinobulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS) are characterized by the presence of inclusions or aggregates of proteinaceous materials. This is due to the generation of aberrant conformations (misfolding) of the proteins responsible for the diseases.
In neuronal cells, the protein quality control (PQC) system may be insufficient to correctly remove the misfolded proteins.
The PQC system requires the activities of efficient chaperones and of the degradative systems ubiquitin-proteasome (UPS) and autophagy. Our approaches are to better characterize the role of the PQC in motoneuron diseases and to find possible ways to boost the PQC activity in order to prevent the damages induced by misfolded proteins to neurons.
Ospite: Alessandro Vercelli