Are synucleinopathies prion-like disorders?
E Angot, JA Steiner, C Hansen, JY Li… - The Lancet …, 2010 - thelancet.com
A shared neuropathological feature of idiopathic Parkinson's disease, dementia with Lewy
bodies, and multiple system atrophy is the development of intracellular aggregates of α …
bodies, and multiple system atrophy is the development of intracellular aggregates of α …
Quantifying prion disease penetrance using large population control cohorts
EV Minikel, SM Vallabh, M Lek, K Estrada… - Science translational …, 2016 - science.org
More than 100,000 genetic variants are reported to cause Mendelian disease in humans,
but the penetrance—the probability that a carrier of the purported disease-causing genotype …
but the penetrance—the probability that a carrier of the purported disease-causing genotype …
Prion-like disorders: blurring the divide between transmissibility and infectivity
M Cushman, BS Johnson, OD King… - Journal of cell …, 2010 - journals.biologists.com
Prions are proteins that access self-templating amyloid forms, which confer phenotypic
changes that can spread from individual to individual within or between species. These …
changes that can spread from individual to individual within or between species. These …
Design and construction of diverse mammalian prion strains
Prions are infectious proteins that encipher biological information within their conformations;
variations in these conformations dictate different prion strains. Toward elucidating the …
variations in these conformations dictate different prion strains. Toward elucidating the …
Prion hypothesis: the end of the controversy?
C Soto - Trends in biochemical sciences, 2011 - cell.com
Forty-three years have passed since it was first proposed that a protein could be the sole
component of the infectious agent responsible for the enigmatic prion diseases. Many …
component of the infectious agent responsible for the enigmatic prion diseases. Many …
The sleep-immunity relationship
CE Gamaldo, AK Shaikh, JC McArthur - Neurologic clinics, 2012 - Elsevier
Research models show a strong interrelationship between sleep quality and immune
function. The proinflammatory cytokines, interleukin-1, interleukin-6, and tumor necrosis …
function. The proinflammatory cytokines, interleukin-1, interleukin-6, and tumor necrosis …
High-resolution structure of infectious prion protein: the final frontier
R Diaz-Espinoza, C Soto - Nature structural & molecular biology, 2012 - nature.com
Prions are the proteinaceous infectious agents responsible for the transmission of prion
diseases. The main or sole component of prions is the misfolded prion protein (PrPSc) …
diseases. The main or sole component of prions is the misfolded prion protein (PrPSc) …
Fatal familial insomnia and sporadic fatal insomnia
L Cracco, BS Appleby, P Gambetti - Handbook of clinical neurology, 2018 - Elsevier
Fatal familial insomnia (FFI) and sporadic fatal insomnia (sFI), or thalamic form of sporadic
Creutzfeldt–Jakob disease MM2 (sCJDMM2T), are prion diseases originally named and …
Creutzfeldt–Jakob disease MM2 (sCJDMM2T), are prion diseases originally named and …
Genetic Creutzfeldt–Jakob disease and fatal familial insomnia: insights into phenotypic variability and disease pathogenesis
S Capellari, R Strammiello, D Saverioni… - Acta …, 2011 - Springer
Human prion diseases are a group of rare neurodegenerative disorders characterized by
the conversion of the constitutively expressed prion protein, PrP C, into an abnormally …
the conversion of the constitutively expressed prion protein, PrP C, into an abnormally …