Results 131 to 140 of about 3,400,014 (272)

A novel, resistance-linked ovine PrP variant and its equivalent mouse variant modulate the in vitro cell-free conversion of rPrP to PrPres [PDF]

, 2006
Prion diseases are associated with the conversion of the normal cellular prion protein, PrPC, to the abnormal, disease-associated form, PrPSc. This conversion can be mimicked in vitro by using a cell-free conversion assay. It has recently been shown that
Kirby, Louise, Manson, Jean C., Houston, Fiona, Goldmann, Wilfred, Gill, Andrew C.   +4 more
core   +1 more source

Interlaboratory performance testing on EPIC v2.0 CNS tumor profiling demonstrates high reproducibility of tumor classification but reveals the need for harmonized copy number variation reporting

Brain Pathology, EarlyView.
Using the Infinium MethylationEPIC v2.0 array and Heidelberg Brain Tumor Classifier v12.8, 24 international laboratories achieved highly reproducible CNS tumor classification (97.9% correct; median β‐correlation r = 0.99), while copy number variation interpretation showed substantial interlaboratory variability, highlighting the need for harmonized CNV
Katrin Mauch‐Mücke, Christin Siewert, Lora Dimitrova, Sandra Pohl, Brendan Osberg, Annika K. Wefers, Julia Lorenz, Tanja Rothhammer‐Hampl, Till Acker, Alonso Barrantes‐Freer, Jean Boutonnat, Bérengère Dadone‐Montaudié, Claire Delbridge, Wolfgang Dietmaier, Sebastian Dintner, Hildegard Dohmen, Matthias Dottermusch, Daniel Erny, Katrin B. M. Frauenknecht, Tobias Goschzik, Patrick N. Harter, Daniela Hirsch, Yanghao Hou, Theo F. J. Kraus, Maggie Lee, Friederike Liesche‐Starnecker, Anne Mc Leer, Linea Melchior, Michael Mittelbronn, Wolf C. Müller, Shalima S. Nair, Julia E. Neumann, Pitt Niehusmann, Nicole Pfarr, Torsten Pietsch, Marco Prinz, Markus Rechsteiner, Regina Reimann, Laveniya Satgunaseelan, Veronica Skarpeteig, Anne J. Skjulsvik, Karl Sotlar, Isabelle Vanden Bempt, Erik Vassella, Katharina J. Weber, Gabriel Wasinger, Andreas Waha, Karen Zwaenepoel, Felix Sahm, Markus J. Riemenschneider, David Capper   +50 more
wiley   +1 more source

Small critical RNAs in the scrapie agent [PDF]

, 2009
Unconventional infectious agents cause transmissible spongiform encephalopathy (TSE) diseases including scrapie and bovine spongiform encephalopathy (BSE) in animals and Creutzfeldt-Jakob disease in humans. The protein only hypothesis claims that the TSE
Marie-Madeleine Ruchoux, Jean-Philippe Deslys, Nicolas Vignier, Pierre Lebon, Jean-Guy Fournier, Sophie Freire, Emmanuel Comoy, Steve Simoneau   +7 more
core  

Inhibition of group-I metabotropic glutamate receptors protects against prion toxicity.

PLoS Pathogens, 2017
Prion infections cause inexorable, progressive neurological dysfunction and neurodegeneration. Expression of the cellular prion protein PrPC is required for toxicity, suggesting the existence of deleterious PrPC-dependent signaling cascades.
Despoina Goniotaki, Asvin K K Lakkaraju, Amulya N Shrivastava, Pamela Bakirci, Silvia Sorce, Assunta Senatore, Rajlakshmi Marpakwar, Simone Hornemann, Fabrizio Gasparini, Antoine Triller, Adriano Aguzzi   +10 more
doaj   +1 more source

α‐Synuclein strain homogeneity in multiple system atrophy subtypes

Brain Pathology, EarlyView.
Two different subtypes of multiple system atrophy are recognized: MSA‐C and MSA‐P. In this manuscript, Lau et al. investigate whether MSA‐C and MSA‐P are caused by different strains of α‐synuclein aggregates. By performing conformational fingerprinting experiments as well as in vitro and in vivo seeding assays, their findings suggest that both MSA‐C ...
Heather H. C. Lau, Nicholas R. G. Silver, Surabhi Mehra, Raphaella W. L. So, Le yao Li, Alison Mao, Erica Stuart, Cian Schmitt‐Ulms, Bradley T. Hyman, Martin Ingelsson, Joel C. Watts   +10 more
wiley   +1 more source

First Report of Single Nucleotide Polymorphisms (SNPs) of the Leporine Shadow of Prion Protein Gene (SPRN) and Absence of Nonsynonymous SNPs in the Open Reading Frame (ORF) in Rabbits

Animals
Prion disorders are fatal infectious diseases that are caused by a buildup of pathogenic prion protein (PrPSc) in susceptible mammals. According to new findings, the shadow of prion protein (Sho) encoded by the shadow of prion protein gene (SPRN) is ...
Sameeullah Memon, Zerui Wang, Wen-Quan Zou, Yong-Chan Kim, Byung-Hoon Jeong   +4 more
doaj   +1 more source

Molecular dynamics simulations elucidate the misfolding mechanisms of secretion‐defective pancreatic lipase variants

The FEBS Journal, EarlyView.
Misfolding mutations in pancreatic lipase have been identified as potential contributors of chronic pancreatitis, an inflammatory disease of the human pancreas. Here, we describe the effect of these misfolding mutations on pancreatic lipase structure using molecular dynamics simulations and structural modeling.
Gyula Hoffka, András Szabó
wiley   +1 more source

Constrictions and shear stress are key determinants of amyloidogenic light chain (AL) amyloidosis

The FEBS Journal, EarlyView.
In this study, we investigate how flow constrictions and shear stress trigger cardiac antibody light chain (AL) fibril formation and deposition. We mimic the mechanical forces exerted by the heart and the microvasculature network using a mini‐peristaltic pump setup and a grid‐type microchannel network, respectively.
Yuji Goto, Keiichi Yamaguchi, Kichitaro Nakajima, Ikuko Yumen, Ruiming He, Hirokazu Matsuda, Tetsuichi Wazawa, Takeharu Nagai, Nobuhiro Tsukada, Suguru Yamamoto, Johannes Buchner, Hirotsugu Ogi   +11 more
wiley   +1 more source

Bone Grafts: Everything You Need to Know

Journal of Periodontal Research, EarlyView.
This review classifies bone grafts (autograft, allograft, xenograft, synthetic) by source and osteogenic/inductive/conductive potential, linking material properties and mechanisms to clinical indications in periodontal and dental regeneration. Key challenges—limited osteoinduction, poor vascularisation, unbalanced resorption, handling difficulties, and
Håvard Jostein Haugen, Javier Sanz, Giuseppe Perale, Augustine Mark Saiz, Mario Romandini, Maryam Rahmati   +5 more
wiley   +1 more source

Novel murine closed‐loop auditory stimulation paradigm elicits macrostructural sleep benefits in neurodegeneration

Journal of Sleep Research, Volume 34, Issue 2, April 2025.
Summary Boosting slow‐wave activity (SWA) by modulating slow waves through closed‐loop auditory stimulation (CLAS) might provide a powerful non‐pharmacological tool to investigate the link between sleep and neurodegeneration. Here, we established mouse CLAS (mCLAS)‐mediated SWA enhancement and explored its effects on sleep deficits in neurodegeneration,
Inês Dias, Sedef Kollarik, Michelle Siegel, Christian R. Baumann, Carlos G. Moreira, Daniela Noain   +5 more
wiley   +1 more source