Neurodegeneration and Clinical Disease in Prion-Infected Mice Oral Treatment Targeting the Unfolded Protein Response Prevents

possibly other neurodegenerative diseases as well. data suggest that the UPR may represent a new therapeutic target for drug development to treat prion disease and emergence of clinical disease in prion-infected mice, whereas untreated animals all succumbed to disease. These pharmacological inhibition would be neuroprotective. The compound prevented neurodegeneration and the used a newly described specific inhibitor of a key UPR kinase mediating translational shutdown to test if long-term translational inhibition, causing a critical decline in key proteins needed for neuronal survival. The authors misfolded prion protein in mice with prion disease causes sustained overactivation of this pathway. This results in . now report that the buildup of et al load in a number of ways including transiently switching off translation. Moreno dealing with misfolded proteins called the unfolded protein response (UPR). The UPR deals with the misfolded protein brains of patients with these neurodegenerative diseases show overactivation of a cellular defense pathway for misfolded disease-specific proteins in the brain, leading to neuronal loss, which is ultimately fatal. In addition, the Parkinson's disease (PD), and prion disease. These diseases share common features, including the accumulation of There are no effective treatments for neurodegenerative disorders such as Alzheimer's disease (AD), Perking Up Prion Disease Therapy