Results 101 to 110 of about 29,749 (223)

Assignment of the Human and Mouse Prion Protein Genes to Homologous Chromosomes [PDF]

, 1986
Purified preparations of scrapie prions contain one major macromolecule, designated prion protein (PrP). Genes encoding PrP are found in normal animals and humans but not within the infectious particles.
Blatt, Cila, Cohn, Vivian H., DeArmond, Stephen J., Fournier, R. E. K., Heinzmann, Camilla, Klisak, Ivana, Lem, Janice, Lucero, Michael, Mohandas, T., Pruisner, Stanley B., Simon, Melvin, Sparkes, Robert S., Weiner, Leslie P., Westaway, David   +13 more
core  

Prion degradation pathways: Potential for therapeutic intervention [PDF]

, 2015
Prion diseases are fatal neurodegenerative disorders. Pathology is closely linked to the misfolding of native cellular PrP(C) into the disease-associated form PrP(Sc) that accumulates in the brain as disease progresses. Although treatments have yet to be
Goold, R, McKinnon, C, Tabrizi, SJ
core   +1 more source

Demyelinating neuropathy as the initial presentation of familial E200K Creutzfeldt–Jakob disease in two patients

Annals of Clinical and Translational Neurology
Objective To describe peripheral neuropathy associated with familial Creutzfeldt‐Jakob disease. Methods We report two unrelated patients with genetic Creutzfeldt–Jakob disease with demyelinating peripheral neuropathy as initial presentation, with a ...
Cécile Delorme, Antoine Pégat, Julian Theuriet, Jean‐Philippe Brandel, Emmanuel Roze, Karine Viala, Julie Zyss, Stéphane Thobois, Anthony Fourier, Emilien Bernard, Juliette Svahn, Chloé Laurencin, Paul Jaulent, Christophe Vandendries, Isabelle Quadrio, Virginie Desestret, David Meyronet, Thierry Maisonobe, Stéphane Haïk, Danielle Seilhean   +19 more
doaj   +1 more source

Neurofilament light chain and tau concentrations are markedly increased in the serum of patients with sporadic Creutzfeldt-Jakob disease, and tau correlates with rate of disease progression [PDF]

, 2018
OBJECTIVES: A blood-based biomarker of neuronal damage in sporadic Creutzfeldt-Jakob disease (sCJD) will be extremely valuable for both clinical practice and research aiming to develop effective therapies.
Collinge, J, Heslegrave, AJ, Jackson, GS, Luk, C, Mead, SH, Thompson, AGB, Zetterberg, H   +6 more
core   +1 more source

Rapidly progressive dementia: probable sporadic Creutzfeldt–Jakob disease in a Yoruba Nigerian woman with rapidly progressive dementia: a case report

Journal of Medical Case Reports
Background Creutzfeldt–Jakob disease is a neurodegenerative disorder that can present with neuropsychiatric features such as dementia; it is a rare cause of rapidly progressive dementia.
A. O. Idowu, A. F. Ogunmodede, A. A. Sanusi, U. C. Eke, S. A. Oyedele, M. B. Fawale, M. A. Komolafe   +6 more
doaj   +1 more source

Efficient transmission and characterization of creutzfeldt-jakob disease strains in bank voles.

PLoS Pathogens, 2006
Transmission of prions between species is limited by the "species barrier," which hampers a full characterization of human prion strains in the mouse model.
Romolo Nonno, Michele A Di Bari, Franco Cardone, Gabriele Vaccari, Paola Fazzi, Giacomo Dell'Omo, Claudia Cartoni, Loredana Ingrosso, Aileen Boyle, Roberta Galeno, Marco Sbriccoli, Hans-Peter Lipp, Moira Bruce, Maurizio Pocchiari, Umberto Agrimi   +14 more
doaj   +2 more sources

Constant Transmission Properties of Variant Creutzfeldt-Jakob Disease in 5 Countries [PDF]

, 2012
Variant Creutzfeldt-Jakob disease (vCJD) has been reported in 12 countries. We hypothesized that a common strain of agent is responsible for all vCJD cases, regardless of geographic origin.
Abigail B. Diack, Bird, Brandel, Bruce, Bruce, Bruce, Chadeau-Hyam, Cornelia Van Duijn, Diane Ritchie, Dickinson, Ermias D. Belay, Fabrizio Tagliavini, Fraser, Head, Hill, Jean C. Manson, Jean-Philippe Brandel, Lawrence B. Schonberger, Matthew Bishop, Pedro Piccardo, Pierluigi Gambetti, Ritchie, Robert G. Will, Sanchez-Juan, Stephane Haik, Victoria Pinion, Will   +26 more
core   +5 more sources

Wariant Choroby Creutzfeldta-Jakoba: aktualny stan wiedzy

Aktualności Neurologiczne, 2011
Choroby wywoływane przez priony obejmują: chorobę Creutzfeldta-Jakoba (Creutzfeldt-Jakob disease, CJD), kuru, chorobę Gerstmanna-Sträusslera-Scheinkera (GSS), śmiertelną rodzinną bezsenność (fatal familial insomnia, FFI) u człowieka, scrapie (polska ...
James W. Ironside
doaj  

MRI and clinical syndrome in dura materrelated Creutzfeldt-Jakob disease [PDF]

, 2018
Objective : Iatrogenic Creutzfeldt-Jakob disease (iCJD) is mainly associated with dura mater (DM) grafts and administration of human growth hormones (hGH). Data on disease course in DM-CJD are limited.
Barsic, B., Eigenbrod, S., Gelpi, E., Heinemann, U., Kallenberg, K., Knauth, M., Krasnianski, A., Kretzschmar, H., Meissner, B., Ramljak, S., Sanchez-Juan, P., Schulz-Schaeffer, W., Zerr, I.   +12 more
core  

Sporadic Creutzfeldt–Jakob disease

, 2018
Sporadic Creutzfeldt-Jakob disease (CJD), the most common human prion disease, is generally regarded as a spontaneous neurodegenerative illness, arising either from a spontaneous PRNP somatic mutation or a stochastic PrP structural change. Alternatively, the possibility of an infection from animals or other source remains to be completely ruled out ...
Zerr, Inga, Parchi, Piero
openaire   +4 more sources