Complex proteinopathies and neurodegeneration: insights from the study of transmissible spongiform encephalopathies

摘要: Protein misfolding diseases are usually associated with deposits of single "key" proteins that somehow drive the pathology; β-amyloid and hyperphosphorylated tau accumulate in Alzheimer's disease, α-synuclein Parkinson's or abnormal prion protein (PrPTSE) transmissible spongiform encephalopathies (TSEs diseases). However, some more than two same brain. These might be considered "complex" proteinopathies. We have studied models TSEs (to explore PrPTSE "secondary proteins") infecting different strains doses TSE agent, factors control incubation period, duration illness histopathology. Model allowed us to investigate whether features histopathology independent appear as a secondary result PrPTSE. Better understanding complex proteinopathies may help explain wide spectrum degenerative why overlap clinically histopathologically. studies also improve diagnosis eventually even suggest new treatments for human neurodegenerative diseases.