Results 131 to 140 of about 73,750 (253)

Human prion diseases

Annals of Medicine, 2000
The term 'prion diseases' refers to a group of neurodegenerative disorders thought to be caused by prions, pathogenic agents with novel modes of replication and transmission. Prion diseases are characterized by long incubation periods ranging from months to years and are invariably fatal once clinical symptoms have appeared.
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Prions And Prion Diseases

African Journal of Clinical and Experimental Microbiology, 2008
A prion is a small infectious particle, which resist inactivation by procedures that modify nucleic acids. Transmissible spongiform encephalopathies (TSEs also known as prion diseases) are a group of progressive conditions that affects the brain and nervous system of humans and animals and are transmitted by prions.
Obi, R K, Nwanebu, F C
openaire   +3 more sources

The L108I polymorphism in mouse prion protein drives spontaneous disease and enhances transmission of atypical and classical prion strains

Brain Pathology, EarlyView.
A single amino acid change (L108I) combined with PrP overexpression drives spontaneous atypical prion formation in mice, enabling also efficient propagation of diverse prion strains. This model allows studying how spontaneous prion diseases arise and provides powerful tools for investigating strain emergence, transmission barriers, and mechanisms ...
Hasier Eraña, Enric Vidal, Natalia Fernández‐Borges, Jorge M. Charco, Carlos M. Díaz‐Domínguez, Cristina Sampedro‐Torres‐Quevedo, Josu Galarza‐Ahumada, Eva Fernández‐Muñoz, Maitena San‐Juan‐Ansoleaga, Miguel Ángel Pérez‐Castro, Nuno Gonçalves‐Anjo, Patricia Piñeiro, Samanta Giler, Nora González‐Martín, Nuria L. Lorenzo, Africa Manero‐Azua, Guiomar Perez de Nanclares, Mariví Geijo, Manuel A. Sánchez‐Martín, Jesús R. Requena, Joaquín Castilla   +20 more
wiley   +1 more source

Therapeutic effect of curcumin derivative GT863 on prion-infected mice

Scientific Reports
In prion diseases, the cellular prion protein (PrPC) forms an abnormal, infectious, and disease-causing form known as PrPSc. Inhibition of prion propagation is a key approach for the treatment of these diseases.
Kenta Teruya, Ayumi Oguma, Michiaki Okuda, Sara Iwabuchi, Hiroyuki Konno, Hiroyuki Arai, Yukitsuka Kudo, Hachiro Sugimoto, Katsumi Doh-ura   +8 more
doaj   +1 more source

Redox environment modulates aggregation of ataxin‐3 in vitro — Implications for drug screening of cysteine‐rich proteins

The FEBS Journal, EarlyView.
Redox environment modulates in vitro aggregation of Ataxin‐3, the protein implicated in spinocerebellar ataxia type 3. Reducing conditions stabilize native monomers and prevent aggregation, whereas oxidative conditions promote the formation of non‐native conformers and disulfide‐linked oligomers within the Josephin domain (JD).
Martyna Podlasiak, Martina Sollazzo, Elisa Monaca, Raffaele Sabbatella, Maria Agnese Morando, Oleg Chertkov, Laura Puglisi, Martina Mascellino, Anna Fricano, Sandra Macedo‐Ribeiro, Caterina Alfano   +10 more
wiley   +1 more source

Evolutionary descent of prion genes from a ZIP metal ion transport ancestor [PDF]

, 2009
In the more than 20 years since its discovery, both the phylogenetic origin and cellular function of the prion protein (PrP) have remained enigmatic.
David Westaway, Gerold Schmitt-Ulms, Holger Wille, Joel C. Watts, Sepehr Ehsani   +4 more
core   +1 more source

Molecular dynamics studies on the NMR and X-ray structures of rabbit prion protein wild-type and mutants

, 2013
Prion diseases are invariably fatal and highly infectious neurodegenerative diseases that affect a wide variety of mammalian species such as sheep, goats, mice, humans, chimpanzees, hamsters, cattle, elks, deer, minks, cats, chicken, pigs, turtles, etc ...
Barlow, Baron, Barry, Barry, Barry, Bastian, Bencsik, Bendheim, Bett, Biswas, Bitel, Bjorndahl, Bockman, Bode, Brun, Caughey, Chianini, Cho, Chou, Chou, Chou, Chou, Chou, Chou, Chou, Christen, Christen, Christen, Christen, Cong, Courageot, Damberger, Di Martino, Dong, Dupiereux, Farquhar, Fernandez-Funez, Fernandez-Funez, Fernandez-Funez, Fernández-Borges, Friedman-Levi, Gabizon, Gabizon, Gao, Garrec, Garssen, Gerber, Gilch, Golanska, Gossert, Groschup, Groschup, Groschup, Guiroy, Handisurya, Hanoux, Hashimoto, Hay, Huang, Ikegami, Ishiguro, Ishikawa, Jackman, Jiapu Zhang, Julien, Kabsch, Kascsak, Kascsak, Kelker, Khan, Kim, Kirby, Kirkwood, Kitamoto, Kocisko, Komar, Korth, Laude, Lawson, Li, Li, Loftus, Lopez, Lührs, Ma, Madec, Mays, Meli, Merz, Miller, Nisbet, Oboznaya, Onodera, Polymenidou, Pérez, Riek, Robakis, Roberts, Rossetti, Sachsamanoglou, Sakudo, Schmerr, Senator, Shin, Shinagawa, Sigurdson, Sigurdson, Sigurdson, Stanker, Sweeting, Takahashi, Takahashi, Takekida, Tang, Timmes, Vidal, Vilette, Vol'pina, Vorberg, Wade, Wang, Wen, Wen, Wiley, Xi, Xiao, Yokoyama, Yokoyama, Yost, Yuanli Zhang, Zahn, Zhang, Zhang, Zhang, Zhang, Zhang, Zhao, Zhou, Zhou, Zhou, Zhou, Zhou, Zocche Soprana   +142 more
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Proteostasis of organelles in aging and disease

The FEBS Journal, EarlyView.
Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi, Cansu Doğan, Dunja Petrović, Gülce Perçin, Seda Koyuncu, David Vilchez   +5 more
wiley   +1 more source

Integrity of H1 helix in prion protein revealed by molecular dynamic simulations to be especially vulnerable to changes in the relative orientation of H1 and its S1 flank

, 2009
In the template-assistance model, normal prion protein (PrPC), the pathogenic cause of prion diseases such as Creutzfeldt-Jakob (CJD) in human, Bovine Spongiform Encephalopathy (BSE) in cow, and scrapie in sheep, converts to infectious prion (PrPSc ...
A Aguzzi, A Aguzzi, A Barducci, A Onufriev, C Guilbert, Chih-Yuan Tseng, Chun-Ping Yu, DA Case, DA Harris, E Lindahl, E Malolepsza, F Eghiaian, GJ Sharman, GS Jackson, H. C. Lee, HJC Berendsen, J Castilla, J Watzlawik, J Ziegler, JP Ryckaert, KM Pan, L Calzolai, M Horiuchi, ML DeMarco, ML DeMarco, MP Morrissey, N Guex, P Derreumaux, P Tompa, R Riek, RI Dima, S Megy, S Santini, S Santini, SA Kozin, SB Prusiner, T Muramoto, W Kabsch, WQ Zou, Y Levy, Y Levy   +40 more
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The Role of the Simulation in Supporting Newly Graduated Nurses in Their First 5 Months of Working Transition: Findings From a Mixed‐Method Study

Journal of Clinical Nursing, EarlyView.
ABSTRACT Aims To understand the role of simulation in ensuring the development of the competencies expected by newly graduated register nurses (NGRNs) from the work initiation up to 5 months of transition. Methods Mixed‐method study design. A longitudinal phase employing the Nurse Competence Scale (NCS, from 0 to 100, excellent) to assess the perceived
Maura Mesaglio, Sara Dentice, Luca Grassetti, Illarj Achil, Anna Bernardinis, Davide Caruzzo, Anna Inserra, Irene Mansutti, Elisa Mattiussi, Sandra Menegoz, Tommaso Piani, Elena Vanzo, Stefania Chiappinotto, Alvisa Palese   +13 more
wiley   +1 more source