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Plaque-type dura mater graft-associated Creutzfeldt-Jakob disease: an autopsied case report [PDF]

Prion
Clinicopathologically, dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) presents as either a non-plaque or plaque type. Here, we report an autopsy case of plaque-type dCJD, supported by genetic and biochemical analyses.
Daisuke Tahara, Daichi Yokoi, Nao Tahara, Akio Akagi, Yuichi Riku, Jun Sone, Hiroaki Miyahara, Hirohisa Watanabe, Masahisa Katsuno, Yasushi Iwasaki   +9 more
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

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

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

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

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

Prion protein conversion at two distinct cellular sites precedes fibrillisation

Nature Communications, 2023
The self-templating nature of prions plays a central role in prion pathogenesis and is associated with infectivity and transmissibility. Since propagation of proteopathic seeds has now been acknowledged a principal pathogenic process in many types of ...
Juan Manuel Ribes, Mitali P. Patel, Hazim A. Halim, Antonio Berretta, Sharon A. Tooze, Peter-Christian Klöhn   +5 more
doaj   +1 more source

The Future of Seed Amplification Assays and Clinical Trials

Frontiers in Aging Neuroscience, 2022
Prion-like seeded misfolding of host proteins is the leading hypothesised cause of neurodegenerative diseases. The exploitation of the mechanism in the protein misfolding cyclic amplification (PMCA) and real-time quaking-induced conversion (RT-QuIC ...
Thomas Coysh, Thomas Coysh, Simon Mead, Simon Mead   +3 more
doaj   +1 more source

Case report of homozygous E200D mutation of PRNP in apparently sporadic Creutzfeldt-Jakob disease

BMC Neurology, 2021
Background Inherited prion diseases are rare autosomal dominant disorders associated with diverse clinical presentations. All are associated with mutation of the gene that encodes prion protein (PRNP).
Ahamad Hassan, Tracy Campbell, Lee Darwent, Hans Odd, Alison Green, John Collinge, Simon Mead   +6 more
doaj   +1 more source