Ontology highlight
ABSTRACT:
SUBMITTER: Homma T
PROVIDER: S-EPMC4462021 | biostudies-literature | 2015 Jun
REPOSITORIES: biostudies-literature
Homma Takujiro T Ishibashi Daisuke D Nakagaki Takehiro T Fuse Takayuki T Mori Tsuyoshi T Satoh Katsuya K Atarashi Ryuichiro R Nishida Noriyuki N
Scientific reports 20150610
Prion diseases are fatal neurodegenerative disorders characterized by the accumulation of prion protein (PrP(C)). To date, there is no effective treatment for the disease. The accumulated PrP, termed PrP(Sc), forms amyloid fibrils and could be infectious. It has been suggested that PrP(Sc) is abnormally folded and resistant to proteolytic degradation, and also inhibits proteasomal functions in infected cells, thereby inducing neuronal death. Recent work indicates that the ubiquitin-proteasome sy ...[more]