Results 21 to 30 of about 56,806 (329)

The Standard Scrapie Cell Assay: Development, Utility and Prospects

Viruses, 2015
Prion diseases are a family of fatal neurodegenerative diseases that involve the misfolding of a host protein, PrPC. Measuring prion infectivity is necessary for determining efficacy of a treatment or infectivity of a prion purification procedure; animal
Jacques van der Merwe, Judd Aiken, David Westaway, Debbie McKenzie   +3 more
doaj   +1 more source

Medium-throughput zebrafish optogenetic platform identifies deficits in subsequent neural activity following brief early exposure to cannabidiol and Δ9-tetrahydrocannabinol

Scientific Reports, 2021
In light of legislative changes and the widespread use of cannabis as a recreational and medicinal drug, delayed effects of cannabis upon brief exposure during embryonic development are of high interest as early pregnancies often go undetected.
Richard Kanyo, Md Ruhul Amin, Laszlo F. Locskai, Danika D. Bouvier, Alexandria M. Olthuis, W. Ted Allison, Declan W. Ali   +6 more
doaj   +1 more source

The CNS in inbred transgenic models of 4-repeat Tauopathy develops consistent tau seeding capacity yet focal and diverse patterns of protein deposition

Molecular Neurodegeneration, 2017
Background MAPT mutations cause neurodegenerative diseases such as frontotemporal dementia but, strikingly, patients with the same mutation may have different clinical phenotypes.
Ghazaleh Eskandari-Sedighi, Nathalie Daude, Hristina Gapeshina, David W. Sanders, Razieh Kamali-Jamil, Jing Yang, Beipei Shi, Holger Wille, Bernardino Ghetti, Marc I. Diamond, Christopher Janus, David Westaway   +11 more
doaj   +1 more source

Mimosine functionalized gold nanoparticles (Mimo-AuNPs) suppress β-amyloid aggregation and neuronal toxicity

Bioactive Materials, 2021
Evidence suggests that increased level/aggregation of beta-amyloid (Aβ) peptides initiate neurodegeneration and subsequent development of Alzheimer's disease (AD). At present, there is no effective treatment for AD. In this study, we reported the effects
Bibin G. Anand, Qi Wu, Govindarajan Karthivashan, Kiran P. Shejale, Sara Amidian, Holger Wille, Satyabrata Kar   +6 more
doaj   +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

Anti-prion drug mPPIg5 inhibits PrP(C) conversion to PrP(Sc). [PDF]

, 2013
Prion diseases, also known as transmissible spongiform encephalopathies, are a group of fatal neurodegenerative diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans.
A Ertmer, A Janaszewska, A Taraboulos, AP Perez, AY Yam, B Caughey, B Caughey, B Caughey, B Klajnert, B Klajnert, B Klajnert, B Klajnert, B Klajnert, B Klajnert, DD Pless, Dietmar Appelhans, DR Borchelt, Ina Maja Vorberg, J Masel, J Solassol, James M. McCarthy, Jeremy C. Simpson, Jörg Tatzelt, K Doh-Ura, KA Nishina, KF Winklhofer, KF Winklhofer, M Fischer, M Miesbauer, Mark S. Rogers, Markus Franke, MP McKinley, N Nishida, NR Deleault, P Heegaard, PC Klohn, PJ Bosque, PMH Heegaard, R Klebe, RJ Kascsak, S Ghaemmaghami, S Gilch, S Supattapone, S Supattapone, SA Priola, SB Prusiner, SB Prusiner, Sibeal Waldron, Ulrike K. Resenberger, Y Lim   +49 more
core   +9 more sources

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

Defining the conformational features of anchorless, poorly neuroinvasive prions. [PDF]

PLoS Pathogens, 2013
Infectious prions cause diverse clinical signs and form an extraordinary range of structures, from amorphous aggregates to fibrils. How the conformation of a prion dictates the disease phenotype remains unclear.
Cyrus Bett, Tim D Kurt, Melanie Lucero, Margarita Trejo, Annemieke J Rozemuller, Qingzhong Kong, K Peter R Nilsson, Eliezer Masliah, Michael B Oldstone, Christina J Sigurdson   +9 more
doaj   +1 more source

Human stem cell-derived astrocytes replicate human prions in a PRNP genotype-dependent manner. [PDF]

, 2017
Prions are infectious agents that cause neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD). The absence of a human cell culture model that replicates human prions has hampered prion disease research for decades.
Alibhai, James, Chandran, Siddharthan, Head, Mark W, Ironside, James W, Krejciova, Zuzana, Krencik, Robert, Manson, Jean, Rzechorzek, Nina M, Ullian, Erik M, Zhao, Chen   +9 more
core   +1 more source

Octarepeat region flexibility impacts prion function, endoproteolysis and disease manifestation

EMBO Molecular Medicine, 2015
The cellular prion protein (PrPC) comprises a natively unstructured N‐terminal domain, including a metal‐binding octarepeat region (OR) and a linker, followed by a C‐terminal domain that misfolds to form PrPSc in Creutzfeldt‐Jakob disease.
Agnes Lau, Alex McDonald, Nathalie Daude, Charles E Mays, Eric D Walter, Robin Aglietti, Robert CC Mercer, Serene Wohlgemuth, Jacques van der Merwe, Jing Yang, Hristina Gapeshina, Chae Kim, Jennifer Grams, Beipei Shi, Holger Wille, Aru Balachandran, Gerold Schmitt‐Ulms, Jiri G Safar, Glenn L Millhauser, David Westaway   +19 more
doaj   +1 more source