Results 11 to 20 of about 51,393 (297)

A noninvasive test for human prion disease using hair roots and scalp [PDF]

Scientific Reports
Invasive tests like cerebrospinal fluid (CSF) examination are highly effective for diagnosing human prion disease (HPD). Real-time quaking-induced conversion (RT-QuIC) CSF assay demonstrates 80–90% sensitivity for HPD diagnosis.
Thi-Thu-Trang Dong, Hiroyuki Honda, Akio Akagi, Yasushi Iwasaki, Hitaru Kishida, Tadashi Tsukamoto, Kensaku Kasuga, Hirotsugu Takashima, Tatsuhiro Terada, Kensuke Ikenaka, Takeshi Ikeuchi, Tsuyoshi Mori, Hideki Mochizuki, Kenjiro Ono, Yoshihisa Takiyama, Tsuyoshi Hamaguchi, Hiroyuki Murota, Nobuo Sanjo, Takeshi Fujimoto, Michio Kitayama, Koji Fujita, Motohiro Yukitake, Shinsuke Fujioka, Noriyuki Nishida, Yoshio Tsuboi, Tetsuyuki Kitamoto, Masaki Takao, Masahito Yamada, Hidehiro Mizusawa, Katsuya Satoh   +29 more
doaj   +2 more sources

Comparison of acetone and sodium phosphotungstic acid precipitation for sample enrichment prior to RT-QuIC for the detection of prion disease [PDF]

BMC Research Notes
Transmissible spongiform encephalopathies, or prion diseases, are fatal neurodegenerative diseases that are medically, economically, and ecologically important.
Eric M. Nicholson, Susan E. Veneziano
doaj   +2 more sources

Rapid generation of prion disease models using AAV‐delivered PrP variants in knockout mice [PDF]

Brain Pathology, Volume 36, Issue 4, July 2026.
We developed a rapid AAV‐based system to generate prion disease models in weeks rather than months. Following systemic AAV9P31 delivery of modified PrP to knockout mice, we achieved brain‐wide expression and successful propagation of both classical (RML) and atypical (GSS‐A117V) prion strains.
Maitena San‐Juan‐Ansoleaga, Eva Fernández‐Muñoz, Jorge M. Charco, Enric Vidal, Diego Herrero‐Martínez, Josu Galarza‐Ahumada, Cristina Sampedro‐Torres‐Quevedo, Samanta Giler, Mariví Geijo, Gloria González‐Aseguinolaza, Hasier Eraña, Joaquín Castilla   +11 more
wiley   +2 more sources

Convergent generation of atypical prions in knockin mouse models of genetic prion disease [PDF]

The Journal of Clinical Investigation
Most cases of human prion disease arise due to spontaneous misfolding of WT or mutant prion protein, yet recapitulating this event in animal models has proven challenging. It remains unclear whether spontaneous prion generation can occur within the mouse
Surabhi Mehra, Matthew E.C. Bourkas, Lech Kaczmarczyk, Erica Stuart, Hamza Arshad, Jennifer K. Griffin, Kathy L. Frost, Daniel J. Walsh, Surachai Supattapone, Stephanie A. Booth, Walker S. Jackson, Joel C. Watts   +11 more
doaj   +2 more sources

The PINK1/Parkin pathway of mitophagy exerts a protective effect during prion disease. [PDF]

PLoS ONE
The PINK1/Parkin pathway of mitophagy has been implicated in the pathogenesis of Parkinson's disease. In prion diseases, a transmissible neurodegenerative disease caused by the misfolded and infectious prion protein (PrPSc), expression of both PINK1 and ...
Anne Ward, Forrest Jessop, Robert Faris, Jason Hollister, Daniel Shoup, Brent Race, Catharine M Bosio, Suzette A Priola   +7 more
doaj   +2 more sources

Pros and cons of a prion-like pathogenesis in Parkinson's disease [PDF]

, 2011
Background: Parkinson's disease (PD) is a slowly progressive neurodegenerative disorder which affects widespread areas of the brainstem, basal ganglia and cerebral cortex.
Chapman Joab, Hilker, Ruediger, Brotchie, Jonathan M, Hilker, Rüdiger, Hilker Ruediger, Brotchie, Jonathan M., Joab Chapman, Ruediger Hilker, Chapman, Joab, Jonathan M Brotchie, Brotchie Jonathan M   +10 more
core   +1 more source

ALK1 controls hepatic vessel formation, angiodiversity, and angiocrine functions in hereditary hemorrhagic telangiectasia of the liver

Hepatology, EarlyView., 2022
Hepatic endothelial Alk1 signaling protects from development of vascular malformations while maintaining organ‐specific endothelial differentiation and angiocrine portmanteau of the names Wingless and Int‐1 signaling. Abstract Background and Aims In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with ...
Christian David Schmid, Victor Olsavszky, Manuel Reinhart, Vanessa Weyer, Felix A. Trogisch, Carsten Sticht, Manuel Winkler, Sina W. Kürschner, Johannes Hoffmann, Roxana Ola, Theresa Staniczek, Joerg Heineke, Beate K. Straub, Jens Mittler, Kai Schledzewski, Peter ten Dijke, Karsten Richter, Steven Dooley, Cyrill Géraud, Sergij Goerdt, Philipp‐Sebastian Koch   +20 more
wiley   +1 more source

Prion protein and prion disease at a glance [PDF]

Journal of Cell Science, 2021
ABSTRACT Prion diseases are neurodegenerative disorders caused by conformational conversion of the cellular prion protein (PrPC) into scrapie prion protein (PrPSc). As the main component of prion, PrPSc acts as an infectious template that recruits and converts normal cellular PrPC into its pathogenic, misfolded isoform. Intriguingly, the
Zhu, Caihong, Aguzzi, Adriano
openaire   +3 more sources

Preparation of lyophilized recombinant prion protein for TSE diagnosis by RT-QuIC

BMC Research Notes, 2018
Objective Transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative diseases, often referred as prion diseases. TSEs result from the misfolding of the cellular prion protein (PrPC) into a pathogenic form (PrPSc) that ...
Soyoun Hwang, Trudy Tatum, Semakaleng Lebepe-Mazur, Eric M. Nicholson   +3 more
doaj   +1 more source

Swine-to-Ferret Transmission of Antigenically Drifted Contemporary Swine H3N2 Influenza A Virus Is an Indicator of Zoonotic Risk to Humans

Viruses, 2023
Human-to-swine transmission of influenza A (H3N2) virus occurs repeatedly and plays a critical role in swine influenza A virus (IAV) evolution and diversity. Human seasonal H3 IAVs were introduced from human-to-swine in the 1990s in the United States and
Carine K. Souza, J. Brian Kimble, Tavis K. Anderson, Zebulun W. Arendsee, David E. Hufnagel, Katharine M. Young, Phillip C. Gauger, Nicola S. Lewis, C. Todd Davis, Sharmi Thor, Amy L. Vincent Baker   +10 more
doaj   +1 more source