Results 41 to 50 of about 8,594 (178)
Incidence of infection in Prnp ARR/ARR sheep following experimental inoculation with or natural exposure to classical scrapie. [PDF]
PLoS ONE, 2014 The prion protein gene (Prnp) is highly influential in determining risk and susceptibility of sheep exposed to classical scrapie. Sheep homozygous for alanine at codon 136 and arginine at codons 154 and 171 (ARR/ARR) of the Prnp gene are historically ...
Martin Jeffrey +5 more
doaj +1 more source
TDP‐43 Aggregation: The Healthy‐Toxic Balance of the Prion‐Like Domain
Advanced Science, Volume 13, Issue 40, 17 July 2026.TDP‐43 function relies on a delicate balance between reversible phase‐separated states and irreversible aggregation. Under physiological conditions, TDP‐43 forms dynamic droplets and oligomers that support normal cellular functions. In pathological contexts, this balance shifts toward aberrant aggregation, leading to toxic species.
Luca Zangrando +2 more
wiley +1 more source
HaloTag Fusion Enables Dynamic Analysis of Prion Protein Biosynthesis, Turnover, and Misfolding
ChemBioChem, Volume 27, Issue 11, 15 June 2026.Prion protein (PrP) misfolding underlies fatal neurodegenerative diseases. We developed a HaloTag‐based PrP fusion enabling spatiotemporal labeling of distinct PrP populations in living cells. This system recapitulates native PrP biology, reveals early misfolding events in disease‐associated mutants, and allows mechanistic interrogation of PrP‐lowering
Antonio Masone +2 more
wiley +1 more source
Classic Scrapie in Sheep with the ARR/ARR Prion Genotype in Germany and France
Emerging Infectious Diseases, 2007 In the past, natural scrapie and bovine spongiform encephalopathy (BSE) infections have essentially not been diagnosed in sheep homozygous for the A136R154R171 haplotype of the prion protein.
Martin H. Groschup +12 more
doaj +1 more source
PLoS ONE, 2020 Scrapie, a prion disease of sheep, is highly resistant to conventional deactivation. Numerous methods to deactivate scrapie have been tested in laboratory animal models, and adequate autoclave treatment can reduce or remove the infectivity of some ...
Eric D Cassmann +2 more
doaj +1 more source
Acta Veterinaria, 2021 Scrapie is an infectious neurodegenerative disease affecting the central nervous system of sheep and goats that belongs to transmissible spongiform encephalopathies.
Nešić Slađan +4 more
doaj +1 more source
Sheep scrapie and deer rabies in England prior to 1800
Prion, 2023 Eighteenth-century England witnessed the emergence of two neurological diseases in animals. Scrapie, a transmissible spongiform encephalopathy, is a fatal neurodegenerative disease of sheep and goats that appears in classical and atypical forms.
Anthony Ness +2 more
doaj +1 more source
Exploring and Targeting the Connection of Iron and Copper Homeostasis to Neurodegenerative Diseases
MedComm, Volume 7, Issue 6, June 2026.Iron and copper dyshomeostasis, along with their interactions with key intrinsically disordered proteins (e.g., Aβ, tau, α‐synuclein) have a strong implication in the onset and progression of neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), Prion diseases (PrDs), Huntington's disease (HD), Wilson's disease (WD),
Xin Liu +9 more
wiley +1 more source
European Journal of Neurology, Volume 33, Issue 6, June 2026.Presymptomatic genetic testing for genetic Creutzfeldt‐Jakob disease requires careful balancing of autonomy, psychological preparedness, and potential familial implications. Based on multidisciplinary experience in Israel, we propose a structured framework including pre‐test counseling, psychological appraisal, genetic testing, in‐person results ...
Dror Shir +28 more
wiley +1 more source