Results 61 to 70 of about 37,766 (292)

The Molecular Pathology of Prion Diseases [PDF]

, 2004
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal ...
Herms, Jochen, Kretzschmar, Hans A., Vassallo, Neville   +2 more
core  

A quantitative assessment of the amount of prion diverted to category 1 materials and wastewater during processing [PDF]

, 2013
In this article the development and parameterization of a quantitative assessment is described that estimates the amount of TSE infectivity that is present in a whole animal carcass (bovine spongiform encephalopathy [BSE] for cattle and classical ...
Adkin, Arnold, Arnold, Benestad, Casalone, Comer, Cooper, Cummins, Daly, Det Norske Veritas, Det Norske Veritas, Det Norske Veritas, Det Norske Veritas, Detwiler, Fediaevsky, Fryer, Gale, Gale, Groschup, Groschup, Hadlow, Hadlow, Kimberlin, Prendergast, Prendergast, Simmons, Terry, Warriss, Wells   +28 more
core   +1 more source

SPG4 and Dementia: Expanding the Clinical Spectrum

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Hereditary spastic paraplegia (HSP) is a group of disorders characterized by progressive spasticity and lower limb weakness, with mutations in SPG4/SPAST being the most common cause. Detailed studies and clinical and molecular comparisons across different populations are missing.
Emanuele Panza, Arun Meyyazhagan, Eliseo Picchi, Gustavo Ribas, Ingrid Faber, Ryosuke Miyamoto, Preethi Basavaraju, Paolo Eusebi, Haripriya Kuchi Bhotla, Mario Stasi, Fabrizio Gaudiello, Francesco Patti, Filippo Maria Santorelli, Marcondes Cavalcante França Jr, José Luiz Pedroso, Orlando Graziani Povoas Barsottini, Hélio Afonso Ghizoni Teive, Peter Henry St George‐Hyslop, Toshitaka Kawarai, Antonio Orlacchio   +19 more
wiley   +1 more source

A quantitative assessment of the prion risk associated with wastewater from carcase-handling facilities [PDF]

, 2013
Wastewater from facilities processing livestock that may harbor transmissible spongiform encephalopathies (TSEs) infectivity is permitted under license for application to land where susceptible livestock may have access. Several previous risk assessments
Adkin, Arnold, British Cattle Movement Service, Comer, Cummins, Diringer, Donaldson, Donaldson, European Commission, Gale, Georgsson, Gravenor, Hinckley, Jacquemot, Mazza, McIntyre, Meat and Livestock Commission, Meat and Livestock Commission, Mittal, Ortiz-Pelaez, Peterson, Seidel, Teunis, Thornton, Yamamoto   +24 more
core   +1 more source

A Systematic Comparison of Alpha‐Synuclein Seed Amplification Assays for Increasing Reproducibility

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Seed amplification assays (SAAs) enable ultrasensitive detection of misfolded α‐synuclein across biofluids and tissues. Yet, heterogeneity in protocols limits cross‐study comparability and clinical translation. Here, we review α‐synuclein SAA methods and their performance across various biological matrices.
Manuela Amaral‐do‐Nascimento, Daniela F. Santos, Tuane C. R. G. Vieira, Tiago F. Outeiro   +3 more
wiley   +1 more source

Conformational diversity in purified prions produced in vitro.

PLoS Pathogens, 2023
Prion diseases are caused by misfolding of either wild-type or mutant forms of the prion protein (PrP) into self-propagating, pathogenic conformers, collectively termed PrPSc.
Daniel J Walsh, Abigail M Schwind, Geoffrey P Noble, Surachai Supattapone   +3 more
doaj   +1 more source

Water and the Biology of Prions and Plaques [PDF]

, 2007
This is an attempt to account for the insolubility and/or aggregation of prions and plaques in terms of a model of water consisting of an equilibrium between high density and low density microdomains.
Graham K. Steel, Phillippa M. Wiggins
core   +1 more source

Prions, protein homeostasis, and phenotypic diversity [PDF]

, 2010
Prions are fascinating but often misunderstood protein aggregation phenomena. The traditional association of the mammalian prion protein with disease has overshadowed a potentially more interesting attribute of prions: their ability to create protein ...
Abramova, Aguzzi, Alberti, Alexandrov, Avery, Balch, Beaumont, Bossdorf, Brachmann, Brachmann, Bradley, Brown, Chernoff, Chernoff, Chiti, Coustou, Cox, Cox, Derkatch, Du, Dunker, Eaglestone, Edskes, Edskes, Fernandez-Bellot, Giacomelli, Glover, Green, Grishin, Griswold, Harrison, Hogan, King, Kodali, Kushnirov, Kussell, Kussell, Lancaster, Liebman, Liu, Masel, Mathur, Morimoto, Nakayashiki, Nakayashiki, Namy, Namy, Nemecek, Osherovich, Patel, Patino, Pedersen, Pigliucci, Randal Halfmann, Santoso, Satpute-Krishnan, Schwimmer, Seger, Senechal, Serio, Shorter, Shorter, Si, Simon Alberti, Smukalla, Sondheimer, Stern, Susan Lindquist, Sweeny, Tanaka, Taneja, Tank, Tessier, True, True, Tyedmers, Verstrepen, Volkov, Wickner, Wickner, Wickner, Wilson, Zenthon   +82 more
core   +1 more source

Amyloidogenic Peptide Fragments Designed From Bacterial Collagen‐like Proteins Form Hydrogel

Advanced Functional Materials, EarlyView.
This study identified amyloidogenic sequence motifs in bacterial collagen‐like proteins and exploited these to design peptides that self‐assemble into β‐sheet fibers and form hydrogels. One hydrogel supported healthy fibroblast growth, showing promise for biocompatible materials. Our work demonstrates that bacterial sequences can be harnessed to create
Vamika Sagar, Xuen J Ng, Lukas Lechel, Ashish Chand Rajwar, Hendrik Bargel, Abhishek A. Jalan   +5 more
wiley   +1 more source

Small Extracellular Vesicles from Neural Cells: Physiological and Pathological Roles, and Potential in Neurodegenerative Therapy

Advanced Healthcare Materials, EarlyView.
Neural cell–derived small extracellular vesicles (sEVs) are emerging as pivotal mediators in neurodegenerative diseases, exerting both pathogenic and therapeutic functions. This review synthesizes current evidence on how sEVs from distinct neural cell types regulate neurodegeneration, neuroprotection, biomarker discovery, and targeted drug delivery ...
Muhammad Waqas Salim, Wei Zhang, Lyndsey Collins‐Praino, Yuling Wang, Andrew Care   +4 more
wiley   +1 more source