Results 81 to 90 of about 2,165,687 (231)

A novel protective prion protein variant that colocalizes with kuru exposure. [PDF]

, 2009
BACKGROUND: Kuru is a devastating epidemic prion disease that affected a highly restricted geographic area of the Papua New Guinea highlands; at its peak, it predominantly affected adult women and children of both sexes.
Whittaker, John, Mein, C., Shah, Paresh, Campbell, T., Whitfield, Jerome, Mein, Charles A, Whittaker, J., Alpers, Michael Philip, Hummerich, H., Collinge, J., Hummerich, Holger, Collinge, John, Uphill, J., Poulter, Mark, Beck, Jon, Al-Dujaily, Huda, Mead, S., Al-Dujaily, H., Alpers, Michael P, Beck, J., Verzilli, C., Mead, Simon, Verzilli, Claudio, Shah, P., Uphill, James, Poulter, M., Campbell, Tracy   +26 more
core   +1 more source

Different isoforms of the non-integrin laminin receptor are present in mouse brain and bind PrP [PDF]

, 2003
The prion protein (PrP) plays a central role in prion diseases, and identifying its cellular receptor appears to be of crucial interest. We previously showed in the yeast twohybrid system that PrP interacts with the 37 kDa precursor (LRP) of the high ...
S. Weiss, J.-P. Deslys, Weiss, S., D. Dormont, Simoneau, S., S. Simoneau, Deslys, J.-P., Lasmézas, C., S. Haïk, C. Leucht, Haik, S., Leucht, C., Dormont, D., C. Lasmézas   +13 more
core   +3 more sources

Single-cell transcriptomics unveils molecular signatures of neuronal vulnerability in a mouse model of prion disease that overlap with Alzheimer’s disease

Nature Communications
Understanding why certain neurons are more sensitive to dysfunction and death caused by misfolded proteins could provide therapeutically relevant insights into neurodegenerative disorders.
Jessy A. Slota, Lise Lamoureux, Kathy L. Frost, Babu V. Sajesh, Stephanie A. Booth   +4 more
doaj   +1 more source

Canadian Creutzfeldt-Jakob disease incidence remained stable during the coronavirus disease (COVID-19) pandemic

Frontiers in Neurology
IntroductionHealthcare disruptions imposed by the coronavirus disease (COVID-19) pandemic and possible biological links between SARS-CoV-2 and prion misfolding might influence the prevalence or characteristics of Creutzfeldt-Jakob Disease (CJD).
Jessy A. Slota, Dobrila Todoric, Vanessa Bergeron, Kristen Avery, Clark Phillipson, Dominic M. S. Kielich, Jennifer Myskiw, Lise Lamoureux, Kathy Frost, Sharon L. R. Simon, Ben A. Bailey-Elkin, Stephanie A. Booth, Stephanie A. Booth   +12 more
doaj   +1 more source

Neural Stem Cell Differentiation and Prion Infection

Bio-Protocol, 2014
Prion diseases are transmissible, fatal, neurodegenerative diseases in human and animals. The molecular basis of neurodegeneration in prion diseases is largely unclear. Developing a cellular model capable of monitoring prion-induced cytotoxicity would be
Yoshi Iwamaru, Takato Takenouchi, Hiroshi Kitani   +2 more
doaj   +1 more source

Prion‐Like Protein LENG8‐Mediated Nucleation Drives Stress Granule Assembly

Advanced Science, EarlyView.
LENG8 is a newly identified stress granule (SG) nucleator required for SG assembly. Following stress, nuclear LENG8 granules disassemble, allowing LENG8 to translocate into the cytoplasm and form independent nucleation foci. These foci fuse with canonical early G3BP1/TIA1 seeds via LENG8‐TIA1 binding to drive SG maturation.
Mingxing Zhang, Xin Wang, Yilan Teng, Zhicheng Wu, Jing Fan, Hongwen Zhu, Peng Dai   +6 more
wiley   +1 more source

Prion protein self-peptides modulate prion interactions and conversion [PDF]

, 2009
Background: Molecular mechanisms underlying prion agent replication, converting host-encoded cellular prion protein (PrPC) into the scrapie associated isoform (PrPSc), are poorly understood.
Bossers, A., Fred G van Zijderveld, Priem, J., Drophatie Timmers-Parohi, Rigter, A., Alex Bossers, Alan Rigter, Timmers-Parohi, D., Jan PM Langeveld, van Zijderveld, F.G., Zijderveld, F.G., van, Jan Priem, Langeveld, J.P.M.   +12 more
core   +1 more source

Probing the Energy Landscape of α‐Synuclein Amyloid Fibril Formation by Systematic K‐to‐Q Mutagenesis

Advanced Science, EarlyView.
We use lysine‐to‐glutamine mutations to study the effect of electrostatics on the kinetics and thermodynamics of alpha‐synuclein amyloid fibril formation. We find that mutational effects map on their structural context within fibrils and identify residues that modulate the energy landscape of alpha‐synuclein self‐assembly. Our work outlines a scalable,
Antonin Kunka, Azad Farzadfard, Jacob Aunstrup Larsen, Rasmus Krogh Norrild, Federica Saraceno, Celia Fricke, Hossein Mohammad‐Beigi, Ahmed Sadek, Jonas Folke, Susana Aznar, Alexander K. Buell   +10 more
wiley   +1 more source

Loss of Octarepeats in Two Processed Prion Pseudogenes in the Red Squirrel, Sciurus vulgaris [PDF]

, 2010
The N-terminal region of the mammalian prion protein (PrP) contains an 'octapeptide' repeat which is involved in copper binding. This eight- or nine-residue peptide is repeated four to seven times, depending on the species, and polymorphisms in repeat ...
Rheede, T., Marlinda Hupkes, Timothy T. Kortum, Wilfried W. de Jong, Ole Madsen, de Jong, W.W., Kohlen, W., Madsen, O., Jong, W.W., de, Kortum, T.T., Wouter Kohlen, Teun van Rheede, Hupkes, H.   +12 more
core   +1 more source

Affected Persons in Laboratory Exposures to Human Pathogens and Toxins in Canada, 2016–2024: A Sector‐Specific Analysis

American Journal of Industrial Medicine, EarlyView.
ABSTRACT Background Exposures to human pathogens and toxins in licensed facilities in Canada have been monitored by a federal surveillance system since 2015, yet the affected persons (APs) in these incidents remain uncharacterized. This study comprehensively describes APs, highlighting sector‐specific patterns and trends over time.
Emily F. Tran, Christine Abalos, Antoinette N. Davis, Audrey Gauthier, Samuel Bonti‐Ankomah   +4 more
wiley   +1 more source