Results 41 to 50 of about 97,942 (322)

Complement 3+-astrocytes are highly abundant in prion diseases, but their abolishment led to an accelerated disease course and early dysregulation of microglia

Acta Neuropathologica Communications, 2019
Astrogliosis and activation of microglia are hallmarks of prion diseases in humans and animals. Both were viewed to be rather independent events in disease pathophysiology, with proinflammatory microglia considered to be the potential neurotoxic species ...
K. Hartmann, D. Sepulveda-Falla, Indigo V. L. Rose, C. Madore, Christiane Muth, J. Matschke, O. Butovsky, Shane A. Liddelow, M. Glatzel, S. Krasemann   +9 more
semanticscholar   +1 more source

Syntaxin-6 delays prion protein fibril formation and prolongs the presence of toxic aggregation intermediates

eLife
Prions replicate via the autocatalytic conversion of cellular prion protein (PrPC) into fibrillar assemblies of misfolded PrP. While this process has been extensively studied in vivo and in vitro, non-physiological reaction conditions of fibril formation
Daljit Sangar, Elizabeth Hill, Kezia Jack, Mark Batchelor, Beenaben Mistry, Juan M Ribes, Graham S Jackson, Simon Mead, Jan Bieschke   +8 more
doaj   +1 more source

Sheep scrapie and deer rabies in England prior to 1800

Prion, 2023
Eighteenth-century England witnessed the emergence of two neurological diseases in animals. Scrapie, a transmissible spongiform encephalopathy, is a fatal neurodegenerative disease of sheep and goats that appears in classical and atypical forms.
Anthony Ness, Judd Aiken, Debbie McKenzie   +2 more
doaj   +1 more source

Cellular and Molecular Mechanisms of Prion Disease.

Annual Review of Pathology, 2019
Prion diseases are rapidly progressive, incurable neurodegenerative disorders caused by misfolded, aggregated proteins known as prions, which are uniquely infectious.
C. Sigurdson, Jason C. Bartz, M. Glatzel
semanticscholar   +1 more source

Antisense oligonucleotides extend survival of prion-infected mice

JCI Insight, 2019
Prion disease is a fatal, incurable neurodegenerative disease of humans and other mammals caused by conversion of cellular prion protein (PrPC) into a self-propagating neurotoxic conformer (prions; PrPSc).
G. J. Raymond, H. Zhao, B. Race, L. Raymond, K. Williams, E. Swayze, Samantha Graffam, Jason Le, T. Caron, J. Stathopoulos, R. O’Keefe, L. Lubke, A. Reidenbach, Allison Kraus, S. Schreiber, C. Mazur, D. Cabin, J. Carroll, E. Minikel, H. Kordasiewicz, B. Caughey, S. M. Vallabh   +21 more
semanticscholar   +1 more source

Differentiated cultures of an immortalized human neural progenitor cell line do not replicate prions despite PrPC overexpression

Prion, 2023
Prions are misfolded proteins that accumulate within the brain in association with a rare group of fatal and infectious neurological disorders in humans and animals.
Jessy A. Slota, Xinzhu Wang, Diana Lusansky, Sarah J. Medina, Stephanie A. Booth   +4 more
doaj   +1 more source

Prions and Prion-like Proteins [PDF]

Journal of Biological Chemistry, 2014
Prions are self-replicating protein aggregates and are the primary causative factor in a number of neurological diseases in mammals. The prion protein (PrP) undergoes a conformational transformation leading to aggregation into an infectious cellular pathogen.
openaire   +2 more sources

Prying into the Prion Hypothesis for Parkinson's Disease

Journal of Neuroscience, 2017
In Parkinson's disease, intracellular α-synuclein inclusions form in neurons. We suggest that prion-like behavior of α-synuclein is a key component in Parkinson's disease pathogenesis. Although multiple molecular changes are involved in the triggering of
P. Brundin, R. Melki
semanticscholar   +1 more source

Adult‐Onset Subacute Sclerosing Panencephalitis Presenting With Subacute Cognitive Deficits

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT We describe the case of a 41‐year‐old man diagnosed with adult‐onset subacute sclerosing panencephalitis (SSPE). The patient presented with subacute progressive cognitive deficits and a neuropsychological profile indicating predominant frontoparietal dysfunction. MRI showed only mild parietal‐predominant cerebral atrophy.
Dennis Yeow, Nicola McKern, Vincent Oxenham, Kate Ahmad, Omar Ahmad   +4 more
wiley   +1 more source

Post COVID-19 AA amyloidosis of the kidneys with rapidly progressive renal failure

Prion, 2023
Coronavirus disease 2019 (COVID-19) pandemic has taken the world by a storm, posing a gruelling challenge to the medical fraternity globally. Besides its very high infectivityinfectivity, significant organ dysfunction occurs in critically ill COVID-19 ...
Tajamul H. Mir, Parvaiz A Zargar, Alok Sharma, Bushra Jabeen, Shephali Sharma, M. Omar Parvaiz, Sabah Bashir, Reem Javeed   +7 more
doaj   +1 more source