Results 51 to 60 of about 29,456 (167)

Health professions and risk of sporadic Creutzfeldt- Jakob disease, 1965 to 2010 [PDF]

, 2012
In 2009, a pathologist with sporadic Creutzfeldt- Jakob Disease (sCJD) was reported to the Spanish registry. This case prompted a request for information on health-related occupation in sCJD cases from countries participating in the European Creutzfeldt ...
Alcalde-Cabero, E., Almazán-Isla, J., Brandel, J-P. (Jean-Philippe), Breithaupt, M., Catarino, J., Collins, S.J. (Steven), Haybäck, J., Höftberger, R. (Romana), Kahana, E., Kovacs, G.G. (Gabor), Ladogana, A. (Anna), Mitrová, E. (Eva), Molesworth, A., Nakamura, Y., Pedro-Cuesta, J. (Jesús) de, Pocchiari, M. (Maurizio), Popovic, M., Ruiz-Tovar, M., Taratuto, A., van Duin, C., Will, R.G. (Robert), Yamada, M., Zerr, I. (Inga)   +22 more
core   +2 more sources

Ring trial of 2nd generation RT‐QuIC diagnostic tests for sporadic CJD

Annals of Clinical and Translational Neurology, 2020
Objective Real‐time quaking‐induced conversion (RT‐QuIC) assays detect prion‐seeding activity in a variety of human biospecimens, including cerebrospinal fluid and olfactory mucosa swabs.
Christina D. Orrú, Bradley R. Groveman, Aaron Foutz, Matilde Bongianni, Franco Cardone, Neil McKenzie, Audrey Culeux, Anna Poleggi, Katarina Grznarova, Daniela Perra, Michele Fiorini, Xiaoqin Liu, Anna Ladogana, Marco Sbriccoli, Andrew G. Hughson, Stéphane Haïk, Alison J. Green, Michael D. Geschwind, Maurizio Pocchiari, Jiri G. Safar, Gianluigi Zanusso, Byron Caughey   +21 more
doaj   +1 more source

Intra- and interspecies interactions between prion proteins and effects of mutations and polymorphisms [PDF]

, 2003
Recently, crystallization of the prion protein in a dimeric form was reported. Here we show that native soluble homogenous FLAG-tagged prion proteins from hamster, man and cattle expressed in the baculovirus system are predominantly dimeric.
Bradley R., Campbell T.A., Clark W.W., Cutlip R.C., Edenhofer F., Goldfarb L.G., Krasemann S., Leucht C., Lopez L., Majtenyi C., Scott M.R., Weiss S.   +11 more
core   +1 more source

Young-onset sporadic Creutzfeldt–Jakob disease with atypical phenotypic features: a case report

Journal of Medical Case Reports, 2019
Background Sporadic Creutzfeldt–Jakob disease, with a mean survival of 6 months, is duly considered among the most fatal neurological disorders. Rapidly progressive dementia with multi-axial involvement of the nervous system is the known presentation ...
Durjoy Lahiri, Srimant Pattnaik, Ashwani Bhat, Souvik Dubey, Atanu Biswas, Biman Kanti Roy   +5 more
doaj   +1 more source

Differentiation of ruminant transmissible spongiform encephalopathy isolate types, including bovine spongiform encephalopathy and CH1641 scrapie [PDF]

, 2010
With increased awareness of the diversity of transmissible spongiform encephalopathy (TSE) strains in the ruminant population, comes an appreciation of the need for improved methods of differential diagnosis. Exposure to bovine spongiform encephalopathy (
Bossers, A., Jacobs, J.G., Keulen, L.J.M., van, Langeveld, J.P.M., Sauer, M., Tang, Y.   +5 more
core   +2 more sources

Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]

, 2010
Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Daggett, Valerie, van der Kamp, Marc W
core   +2 more sources

Movement Disorders in Prionopathies: A Systematic Review

Tremor and Other Hyperkinetic Movements, 2019
Background: Movement disorders are frequent features of prionopathies. However, their prevalence and onset remain poorly described. Methods: We performed a systematic review of case reports and case series of pathologically- and genetically confirmed ...
Federico Rodriguez-Porcel, Vinícius Boaratti Ciarlariello, Alok K. Dwivedi, Lilia Lovera, Gustavo Da Prat, Ricardo Lopez-Castellanos, Ritika Suri, Holly Laub, Ruth H. Walker, Orlando Barsottini, José Luiz Pedroso, Alberto J. Espay   +11 more
doaj   +1 more source

Creutzfeldt–Jakob disease: A case report and differential diagnoses

Clinical Case Reports, 2022
Although sporadic Creutzfeldt–Jakob disease is a rare neurodegenerative disease and often difficult to diagnose at the earliest onset, meticulous clinical examination, electroencephalography, and neuroimaging findings will help in diagnosis.
Akash Raut, Anjila Thapa, Ashish Shrestha, Kamal Saud, Reema Rajbhandari, Shailendra Katwal   +5 more
doaj   +1 more source

Molecular dynamics as an approach to study prion protein misfolding and the effect of pathogenic mutations [PDF]

, 2011
Computer simulation of protein dynamics offers unique high-resolution information that complements experiment. Using experimentally derived structures of the natively folded prion protein (PrP), physically realistic dynamics and conformational changes ...
Daggett, Valerie, van der Kamp, Marc W
core   +2 more sources

Emerging infectious diseases: coping with uncertainty [PDF]

, 2009
The world’s scientific community must be in a state of constant readiness to address the threat posed by newly emerging infectious diseases. Whether the disease in question is SARS in humans or BSE in animals, scientists must be able to put into action ...
Cummings, L
core   +1 more source