Results 21 to 30 of about 2,165,687 (231)

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   +2 more sources

A New Cell Model for Investigating Prion Strain Selection and Adaptation

Viruses, 2019
Prion diseases are fatal neurodegenerative diseases that affect humans and animals. Prion strains, conformational variants of misfolded prion proteins, are associated with distinct clinical and pathological phenotypes.
Alexandra Philiastides, Juan Manuel Ribes, Daniel Chun-Mun Yip, Christian Schmidt, Iryna Benilova, Peter-Christian Klöhn   +5 more
doaj   +1 more source

Genetic risk factors for Creutzfeldt-Jakob disease

Neurobiology of Disease, 2020
Prion diseases are a group of fatal neurodegenerative disorders of mammals that share a central role for prion protein (PrP, gene PRNP) in their pathogenesis.
Emma Jones, Simon Mead
doaj   +1 more source

Combination of structure-based virtual screening, molecular docking and molecular dynamics approaches for the discovery of anti-prion fibril flavonoids

Frontiers in Molecular Biosciences, 2023
Prion diseases are a group of rare neurodegenerative diseases caused by the structural conversion of cellular prion into Scrapie prion resulting aggregated fibrils. Therapy of prion diseases has been developed for several decades, especially drug designs
Cheng-Ping Jheng, Cheng-I Lee, Cheng-I Lee, Cheng-I Lee   +3 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.
Jeremy C. Simpson (29225), Franke, Markus, Sibeal Waldron (277507), Appelhans Dietmar, Rogers Mark S., Dietmar Appelhans (277509), Resenberger, Ulrike K., Simpson Jeremy C., Simpson, Jeremy C., Jörg Tatzelt, Franke Markus, Resenberger Ulrike K., Rogers, Mark S., McCarthy James M., Mark S. Rogers (277510), James M McCarthy, Sibeal Waldron, Dietmar Appelhans, Appelhans, Dietmar, Mark S Rogers, Tatzelt Jörg, Tatzelt, Jörg, Ulrike K. Resenberger (277506), McCarthy, James M., Waldron, Sibeal, Markus Franke, Ulrike K Resenberger, Markus Franke (277505), Waldron Sibeal, Jeremy C Simpson, Jörg Tatzelt (277508), James M. McCarthy (277504)   +31 more
core   +2 more sources

Intra- and interspecies interactions between prion proteins and effects of mutations and polymorphisms [PDF]

, 2003
Recently, crystallization of the prion protein in a dimeric form was reported. Here we show that native soluble homogenous FLAG-tagged prion proteins from hamster, man and cattle expressed in the baculovirus system are predominantly dimeric.
Hundt, C., Weiss, S., Riley, M. L., Gauczynski, S., Leucht, C.   +4 more
core   +1 more source

Characterization of the prion protein in relation to normal cellular function and in disease [PDF]

, 2012
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of rare and fatal neurodegenerative disorders that can affect both human and animals.
Wik, Lotta
core   +1 more source

Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]

, 2010
Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Valerie Daggett, Daggett, Valerie, van der Kamp, Marc W, Marc W. van der Kamp   +3 more
core   +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