Results 1 to 10 of about 49,299 (116)

A Neuronal Cell Line Model for Studying Camel Prions [PDF]

Pathogens
Prion diseases are fatal neurodegenerative disorders that affect humans and animals, caused by the conformational conversion of the normal cellular prion protein (PrPC) into its misfolded, infectious isoform PrPSc.
Basant Abdulrahman, Shabboo Rahimi Aqdam, Matteo Mosca, Hanaa Ahmed-Hassan, Melissa Razcon-Echeagaray, Lia Popa, Sabine Gilch, Baaissa Babelhadj, Gabriele Vaccari, Hermann M. Schätzl   +9 more
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Palliative care symptoms of people living with rapidly progressive prion diseases: a systematic review [PDF]

BMC Palliative Care
Background Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal dementia with an average prognosis of four to six months. Palliative care, as a holistic person-centred approach to supporting people and families affected by life-limiting ...
Rachel T. Williams, Nicola White, Joshua Kearns, Kirsty McNiven, Ann Rochelle Marasigan, Elizabeth L. Sampson, Catherine J. Evans, Simon Mead, Nuriye Kupeli, the EMBED‐Care Programme   +9 more
doaj   +2 more sources

In memoriam of Pawel P. Liberski (1954–2025) [PDF]

Prion
In memoriam of Pawel P. Liberski, an enthusiastic scientist of rare intelligence, a loyal and generous friend, and a truly vivid personality.
Beata Sikorska
doaj   +2 more sources

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

Bank vole prion protein extends the use of RT-QuIC assays to detect prions in a range of inherited prion diseases

Scientific Reports, 2021
The cerebrospinal fluid (CSF) real-time quaking-induced conversion assay (RT-QuIC) is an ultrasensitive prion amyloid seeding assay for diagnosis of sporadic Creutzfeldt–Jakob disease (CJD) but several prion strains remain unexplored or resistant to ...
Tze How Mok, Akin Nihat, Connie Luk, Danielle Sequeira, Mark Batchelor, Simon Mead, John Collinge, Graham S. Jackson   +7 more
doaj   +1 more source

A high-content neuron imaging assay demonstrates inhibition of prion disease-associated neurotoxicity by an anti-prion protein antibody

Scientific Reports, 2022
There is an urgent need to develop disease-modifying therapies to treat neurodegenerative diseases which pose increasing challenges to global healthcare systems.
Madeleine Reilly, Iryna Benilova, Azadeh Khalili-Shirazi, Christian Schmidt, Parvin Ahmed, Daniel Yip, Parmjit S. Jat, John Collinge   +7 more
doaj   +1 more source

Prion protein gene mutation detection using long-read Nanopore sequencing

Scientific Reports, 2022
Prion diseases are fatal neurodegenerative conditions that affect humans and animals. Rapid and accurate sequencing of the prion gene PRNP is paramount to human prion disease diagnosis and for animal surveillance programmes.
François Kroll, Athanasios Dimitriadis, Tracy Campbell, Lee Darwent, John Collinge, Simon Mead, Emmanuelle Vire   +6 more
doaj   +1 more source

2.7 Å cryo-EM structure of ex vivo RML prion fibrils

Nature Communications, 2022
High-resolution structures of mammalian prions have remained elusive. Here, Manka et al. report the cryo-EM structure of infectious RML prion fibrils from mice.
Szymon W. Manka, Wenjuan Zhang, Adam Wenborn, Jemma Betts, Susan Joiner, Helen R. Saibil, John Collinge, Jonathan D. F. Wadsworth   +7 more
doaj   +1 more source

Correction [PDF]

Prion
doaj   +2 more sources

Essential Components of Synthetic Infectious Prion Formation De Novo

Biomolecules, 2022
Prion diseases are a class of neurodegenerative diseases that are uniquely infectious. Whilst their general replication mechanism is well understood, the components required for the formation and propagation of highly infectious prions are poorly ...
Kezia Jack, Graham S. Jackson, Jan Bieschke   +2 more
doaj   +1 more source