Results 1 to 10 of about 5,604,304 (240)

Distribution of Misfolded Prion Protein Seeding Activity Alone Does Not Predict Regions of Neurodegeneration.

PLoS Biology, 2016
Protein misfolding is common across many neurodegenerative diseases, with misfolded proteins acting as seeds for "prion-like" conversion of normally folded protein to abnormal conformations.
James Alibhai, Richard A Blanco, Marcelo A Barria, Pedro Piccardo, Byron Caughey, V Hugh Perry, Tom C Freeman, Jean C Manson   +7 more
doaj   +2 more sources

Prion protein E219K polymorphism: from the discovery of the KANNO blood group to interventions for human prion disease [PDF]

Frontiers in Neurology
KANNO is a new human blood group that was recently discovered. The KANNO antigen shares the PRNP gene with the prion protein and the prion protein E219K polymorphism determines the presence or absence of the KANNO antigen and the development of anti ...
Si-Si Wang, Zhao-Li Meng, Yi-Wen Zhang, Yi-Shuang Yan, Ling-Bo Li   +4 more
doaj   +2 more sources

Genetic aspects of human prion diseases

Frontiers in Neurology, 2022
Human prion diseases are rapidly progressive and fatal neurodegenerative conditions caused by a disease-causing isoform of the native prion protein. The prion protein gene (PRNP) encodes for the cellular prion protein, which is the biological substrate ...
Brian S. Appleby, Brian S. Appleby, Shashirekha Shetty, Shashirekha Shetty, Mohamed Elkasaby   +4 more
doaj   +1 more source

Oral administration of repurposed drug targeting Cyp46A1 increases survival times of prion infected mice

Acta Neuropathologica Communications, 2021
Prion diseases are fatal, infectious, and incurable neurodegenerative disorders caused by misfolding of the cellular prion protein (PrPC) into the infectious isoform (PrPSc). In humans, there are sporadic, genetic and infectious etiologies, with sporadic
Tahir Ali, Samia Hannaoui, Satish Nemani, Waqas Tahir, Irina Zemlyankina, Pearl Cherry, Su Yeon Shim, Valerie Sim, Hermann M. Schaetzl, Sabine Gilch   +9 more
doaj   +1 more source

Anti-Prion Systems in Saccharomyces cerevisiae Turn an Avalanche of Prions into a Flurry

Viruses, 2022
Prions are infectious proteins, mostly having a self-propagating amyloid (filamentous protein polymer) structure consisting of an abnormal form of a normally soluble protein.
Moonil Son, Reed B. Wickner
doaj   +1 more source

Allelic Interference in Prion Replication Is Modulated by the Convertibility of the Interfering PrP ^C and Other Host-Specific Factors

mBio, 2021
Prion propagation can be interfered with by the expression of a second prion protein in the host. In the present study, we investigated prion propagation in a host expressing two different prion protein genes.
Juan Carlos Espinosa, Olivier Andreoletti, Alba Marín-Moreno, Severine Lugan, Patricia Aguilar-Calvo, Hervé Cassard, Patricia Lorenzo, Jean-Yves Douet, Ana Villa-Díaz, Naima Aron, Irene Prieto, Alvina Huor, Juan María Torres   +12 more
doaj   +1 more source

Prion protein lowering is a disease-modifying therapy across prion disease stages, strains and endpoints

bioRxiv, 2020
Lowering of prion protein (PrP) expression in the brain is a genetically validated therapeutic hypothesis in prion disease. We recently showed that antisense oligonucleotide (ASO)-mediated PrP suppression extends survival and delays disease onset in ...
E. Minikel, H. Zhao, Jason Le, J. O’Moore, Rose Pitstick, Samantha Graffam, G. Carlson, M. Kavanaugh, J. Kriz, J. B. Kim, Jiyan Ma, H. Wille, J. Aiken, D. McKenzie, K. Doh-ura, M. Beck, R. O’Keefe, J. Stathopoulos, T. Caron, S. Schreiber, J. Carroll, H. Kordasiewicz, D. Cabin, S. M. Vallabh   +23 more
semanticscholar   +1 more source

Treatment of Prion Disease with Heterologous Prion Proteins. [PDF]

PLoS ONE, 2015
Prion diseases such as Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy in cattle, and scrapie in sheep are fatal neurodegenerative diseases for which there is no effective treatment.
Pamela J Skinner, Hyeon O Kim, Damani Bryant, Nikilyn J Kinzel, Cavan Reilly, Suzette A Priola, Anne E Ward, Patricia A Goodman, Katherine Olson, Davis M Seelig   +9 more
doaj   +1 more source

Overexpression of mouse prion protein in transgenic mice causes a non-transmissible spongiform encephalopathy

Scientific Reports, 2022
Transgenic mice over-expressing human PRNP or murine Prnp transgenes on a mouse prion protein knockout background have made key contributions to the understanding of human prion diseases and have provided the basis for many of the fundamental advances in
Graham S. Jackson, Jacqueline Linehan, Sebastian Brandner, Emmanuel A. Asante, Jonathan D. F. Wadsworth, John Collinge   +5 more
doaj   +1 more source

DNA methylation analysis on purified neurons and glia dissects age and Alzheimer’s disease-specific changes in the human cortex

Epigenetics & Chromatin, 2018
Background Epigenome-wide association studies (EWAS) based on human brain samples allow a deep and direct understanding of epigenetic dysregulation in Alzheimer’s disease (AD).
Gilles Gasparoni, Sebastian Bultmann, Pavlo Lutsik, Theo F. J. Kraus, Sabrina Sordon, Julia Vlcek, Vanessa Dietinger, Martina Steinmaurer, Melanie Haider, Christopher B. Mulholland, Thomas Arzberger, Sigrun Roeber, Matthias Riemenschneider, Hans A. Kretzschmar, Armin Giese, Heinrich Leonhardt, Jörn Walter   +16 more
doaj   +1 more source