Results 61 to 70 of about 78,455 (315)

Clinical–Radiological Spectrum of Cerebral Amyloid Angiopathy‐Related Inflammation

Annals of Neurology, EarlyView.
Objective To identify clinical and radiological features of cerebral amyloid angiopathy‐related inflammation (CAA‐ri), and compare these features with those of sporadic CAA, to improve the understanding, diagnosis, and clinical care of CAA‐ri. Methods We retrospectively reviewed routine clinical data from 37 patients with CAA‐ri and 158 patients with ...
Larysa Panteleienko, Gargi Banerjee, Dermot Mallon, Kitti Thiankhaw, Rupert Oliver, Victoria Harvey, Gareth Ambler, Michael Zandi, Hans Rolf Jäger, David J. Werring   +9 more
wiley   +1 more source

Stochastic Modelling Approach to the Incubation Time of Prionic Diseases

, 2003
Transmissible spongiform encephalopathies like the bovine spongiform encephalopathy (BSE) and the Creutzfeldt-Jakob disease (CJD) in humans are neurodegenerative diseases for which prions are the attributed pathogenic agents.
A. S. Ferreira, A. L. Horwich, B. J. West, D. J. Stekel, G. C. Telling, J. C. Cressoni, J. Collinge, J. D. Murray, J. S. Griffith, M. A. A. da Silva, M. Eigen, R. G. Will, R. M. Anderson, S. B. Prusiner, S. B. Prusiner, S. B. Prusiner   +15 more
core   +1 more source

Synthesis and structural characterization of a mimetic membrane-anchored prion protein [PDF]

, 2006
During pathogenesis of transmissible spongiform encephalopathies (TSEs) an abnormal form (PrPSc) of the host encoded prion protein (PrPC) accumulates in insoluble fibrils and plaques. The two forms of PrP appear to have identical covalent structures, but
Andrew C. Gill, Ansari AA, Atvinder K. Rullay, Bertozzi CR, Blanch EW, Borchelt D, Caughey B, Caughey B, Caughey B, Caughey BW, Critchley P, David H. Crout, Eberl H, Ferris A, Georges C, Gill AC, Govaerts C, Haire LF, Harris DA, Hornemann S, Ian D. Sylvester, Imanpreet K. Bath, Kazlauskaite J, Kazlauskaite J, Kenyon GL, King SA, Kirby L, Klein TR, Kocienski PJ, Legname G, Matthew R. Hicks, Mui B, Nosjean O, Pan KM, Poss AJ, Safar J, Seddon AM, Shyng SL, Stahl N, Stahl N, Teresa J. T. Pinheiro, Whitesell JK, Wuthrich K, Zahn R, Zhang ZY   +44 more
core   +1 more source

Anatomical Associations Between Focal Mitochondrial Metabolism and Patterns of Neurodegeneration in Amyotrophic Lateral Sclerosis

Annals of Neurology, EarlyView.
Objective Amyotrophic lateral sclerosis (ALS) has a very specific neuroimaging signature, but the molecular underpinnings of the strikingly selective anatomic involvement have not elucidated to date. Accordingly, a large neuroimaging study was conducted with 258 participants to evaluate associations between patterns of neurodegeneration and focal ...
Marlene Tahedl, We Fong Siah, Efstratios Karavasilis, Jennifer C. Hengeveld, Mark A. Doherty, Russell L. McLaughlin, Orla Hardiman, Ee Ling Tan, Foteini Christidi, Jana Kleinerova, Peter Bede   +10 more
wiley   +1 more source

Seeding and transgenic overexpression of alpha‐synuclein triggers dendritic spine pathology in the neocortex

EMBO Molecular Medicine, 2017
Although misfolded and aggregated α‐synuclein (α‐syn) is recognized in the disease progression of synucleinopathies, its role in the impairment of cortical circuitries and synaptic plasticity remains incompletely understood.
Sonja Blumenstock, Eva F Rodrigues, Finn Peters, Lidia Blazquez‐Llorca, Felix Schmidt, Armin Giese, Jochen Herms   +6 more
doaj   +1 more source

Atypical status of bovine spongiform encephalopathy in Poland: a molecular typing study [PDF]

, 2008
The aim of this study was to analyze molecular features of protease-resistant prion protein (PrPres) in Western blots of BSE cases diagnosed in Poland with respect to a possible atypical status.
Jacobs, J.G., Langeveld, J.P.M., Polak, M.P., Zmudzinski, J.F.   +3 more
core   +1 more source

The Phenotypic Spectrum of Sporadic Creutzfeldt‐Jakob Disease Cortical Subtype

Annals of Neurology, EarlyView.
Objective The objective of this study was to characterize the phenotypic spectrum of the rare sporadic Creutzfeldt‐Jakob disease cortical subtype (sCJDMM/MV2C) in a large multicentric autopsy cohort. Methods We evaluated clinical histories, biofluid markers, brain diffusion‐weighted (DW)‐magnetic resonance imaging (MRI), and electroencephalogram (EEG ...
Simone Baiardi, Claudia Marina Vargiu, Brian S. Appleby, Marcelo Barria, Giuseppe Mario Bentivenga, Ignazio Calì, Benedetta Carlà, Mark Cohen, Armin Giese, Jochen Herms, Aino‐Minerva Kortelainen, Anna Ladogana, Angela Mammana, Diane Ritchie, Otto Windl, Sabina Capellari, Piero Parchi   +16 more
wiley   +1 more source

Evidence for Oxidative Stress in Experimental Prion Disease

Neurobiology of Disease, 2000
Oxidative stress has been shown to be important in several neurodegenerative disorders. Previous in vitro studies have already demonstrated the ability of a prion protein fragment to induce oxidative stress in cultured cells.
Marin Guentchev, Till Voigtländer, Christine Haberler, Martin H. Groschup, Herbert Budka   +4 more
doaj   +1 more source

Large-scale lipidomic profiling identifies novel potential biomarkers for prion diseases and highlights lipid raft-related pathways

Veterinary Research, 2021
Prion diseases are transmissible spongiform encephalopathies induced by the abnormally-folded prion protein (PrPSc), which is derived from the normal prion protein (PrPC).
Yong-Chan Kim, Junbeom Lee, Dae-Weon Lee, Byung-Hoon Jeong   +3 more
doaj   +1 more source

Shortest known prion protein allele in highly BSE-susceptible lemurs [PDF]

, 2000
We describe the shortest prion protein allele known to date. Surprisingly, it is found as a polymorphism exactly in a species (prosimian lemurs) which seems highly susceptible to oral infection with BSE-derived prions. The truncation of the prion protein
Gilch, S., Schätzl, H. M., Spielhaupter, C.   +2 more
core   +1 more source