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Prion Diseases [PDF]

Continuum, 2015
This article presents an update on the clinical aspects of human prion disease, including the wide spectrum of their presentations.Prion diseases, a group of disorders caused by abnormally shaped proteins called prions, occur in sporadic (Jakob-Creutzfeldt disease), genetic (genetic Jakob-Creutzfeldt disease, Gerstmann-Sträussler-Scheinker syndrome ...
openaire   +7 more sources

Interspecies transmission to bovinized transgenic mice uncovers new features of a CH1641-like scrapie isolate

Veterinary Research, 2018
In animal prion diseases, including bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease in cervids, and scrapie in sheep and goats, a disease-associated isoform of prion protein (PrPd) accumulates in the brains of affected animals ...
Kohtaro Miyazawa, Kentaro Masujin, Yuichi Matsuura, Yoshifumi Iwamaru, Takashi Yokoyama, Hiroyuki Okada   +5 more
doaj   +1 more source

A Retrospective Cohort Study of a Newly Proposed Criteria for Sporadic Creutzfeldt–Jakob Disease

Diagnostics
Background/Objectives: Sporadic Creutzfeldt–Jakob disease (sCJD) is a fatal neurodegenerative disorder traditionally diagnosed based on the World Health Organization (WHO) criteria in 1998. Recently, Hermann et al.
Toshiaki Nonaka, Ryusuke Ae, Koki Kosami, Hiroya Tange, Miho Kaneko, Takehiro Nakagaki, Tsuyoshi Hamaguchi, Nobuo Sanjo, Yoshikazu Nakamura, Tetsuyuki Kitamoto, Yoshiyuki Kuroiwa, Kensaku Kasuga, Manabu Doyu, Fumiaki Tanaka, Koji Abe, Shigeo Murayama, Ichiro Yabe, Hideki Mochizuki, Takuya Matsushita, Hiroyuki Murai, Masashi Aoki, Koji Fujita, Masafumi Harada, Masaki Takao, Tadashi Tsukamoto, Yasushi Iwasaki, Masahito Yamada, Hidehiro Mizusawa, Katsuya Satoh, Noriyuki Nishida   +29 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

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

Short incubation periods of atypical H-type BSE in cattle with EK211 and KK211 prion protein genotypes after intracranial inoculation

Frontiers in Veterinary Science, 2023
In 2006, a case of atypical H-type BSE (H-BSE) was found to be associated with a germline mutation in the PRNP gene that resulted in a lysine substitution for glutamic acid at codon 211 (E211K). The E211K amino acid substitution in cattle is analogous to
Eric D. Cassmann, Alexis J. Frese, Alexis J. Frese, Alexis J. Frese, Kelsey A. Becker, Kelsey A. Becker, Justin J. Greenlee   +6 more
doaj   +1 more source

Swine influenza A replicon particle and live attenuated influenza virus vaccines induce differential systemic and mucosal antibody and T cell responses

Frontiers in Veterinary Science
Influenza A virus (IAV) in swine is a significant economic concern, and there is a critical need to improve vaccine efficacy. Commercial and experimental vaccine platforms are effective against homologous infection but may not reliably provide protection
Meghan Wymore Brand, Bryan S. Kaplan, Carine K. Souza, Jayne E. Wiarda, J. Brian Kimble, Bailey Arruda, Daniel R. Perez, Amy L. Baker   +7 more
doaj   +1 more source

Evaluation of Antemortem Diagnostic Techniques in Goats Naturally Infected With Scrapie

Frontiers in Veterinary Science, 2020
Scrapie is a naturally occurring transmissible spongiform encephalopathy (TSE) that affects sheep and goats. Sheep and goats can be infected with scrapie as lambs or kids via contact with the placenta or placental fluids, or from ingestion of prions shed
Najiba Mammadova, M. Heather West Greenlee, S. Jo Moore, Soyoun Hwang, Aaron D. Lehmkuhl, Eric M. Nicholson, Justin J. Greenlee   +6 more
doaj   +1 more source

Treatment with KCL‐286, a first‐in‐class retinoic acid receptor‐β (RARβ) agonist, ameliorates neuronal DNA damage and inflammation in a mouse model of Alzheimer's disease

FEBS Open Bio, EarlyView.
Repair of neuronal DNA damage in Alzheimer's disease by KCL‐286. (A) Amyloid‐β oligomers and plaques impair neuronal DNA repair pathways, leading to DNA double‐strand breaks and glial activation. (B) KCL‐286 activates RARβ/RXR signalling via retinoic acid response elements (RAREs), associated with increased BRCA1 expression, enhanced DNA repair and ...
Natasha Hill, Hassan Yousef Othman AlMuallim, Elouisa Maddock, Carl Hobbs, Earl Clarke, Maria B. Goncalves, Jonathan P. T. Corcoran   +6 more
wiley   +1 more source

Aqueous extraction of formalin-fixed paraffin-embedded tissue and detection of prion disease using real-time quaking-induced conversion

BMC Research Notes
Objective The goal of the research presented here is to determine if methods previously developed for the aqueous extraction of PrPSc from formalin-fixed paraffin-embedded tissue (FFPET) are applicable to the detection PrPSc by real-time quaking induced ...
Eric M. Nicholson, Justin J. Greenlee, Soyoun Hwang   +2 more
doaj   +1 more source