Prion diseases are characterized by neurodegeneration and protein aggregation,which are caused by the accumulation of a misfolded and protease-resistant form of the cellular prion protein.Several studies suggest that ...Prion diseases are characterized by neurodegeneration and protein aggregation,which are caused by the accumulation of a misfolded and protease-resistant form of the cellular prion protein.Several studies suggest that the accumulation of a misfolded alternate cellular protein disturbs quality control mechanisms,leading to endoplasmic reticulum(ER)stress involved in prion diseases[1-3].Prion infection activates the splicing of the unfolded protein response transcription factor XBP-1.Misfolded mutant PrP associated with inherited.展开更多
基金supported by the China Mega-Project for Infectious Disease[2018ZX10102001,2018ZX10711001,and 2018ZX10734404]the SKLID Development[2011SKLID104]。
文摘Prion diseases are characterized by neurodegeneration and protein aggregation,which are caused by the accumulation of a misfolded and protease-resistant form of the cellular prion protein.Several studies suggest that the accumulation of a misfolded alternate cellular protein disturbs quality control mechanisms,leading to endoplasmic reticulum(ER)stress involved in prion diseases[1-3].Prion infection activates the splicing of the unfolded protein response transcription factor XBP-1.Misfolded mutant PrP associated with inherited.