Results 61 to 70 of about 16,731 (246)
Oral Transmission of L-Type Bovine Spongiform Encephalopathy Agent among Cattle
Emerging Infectious Diseases, 2017 To determine oral transmissibility of the L-type bovine spongiform encephalopathy (BSE) prion, we orally inoculated 16 calves with brain homogenates of the agent. Only 1 animal, given a high dose, showed signs and died at 88 months. These results suggest
Hiroyuki Okada +7 more
doaj +1 more source
No Adaptation of the Prion Strain in a Heterozygous Case of Variant Creutzfeldt-Jakob Disease
Emerging Infectious Diseases, 2020 We investigated a clinical case of variant Creutzfeldt-Jakob Disease in a person heterozygous for methionine/valine at codon 129 of the prion protein gene and identified the same strain properties in variant Creutzfeldt-Jakob disease in methionine ...
Aileen Boyle +6 more
doaj +1 more source
Shortest known prion protein allele in highly BSE-susceptible lemurs [PDF]
, 2000 We describe the shortest prion protein allele known to date. Surprisingly, it is found as a polymorphism exactly in a species (prosimian lemurs) which seems highly susceptible to oral infection with BSE-derived prions. The truncation of the prion protein
Gilch, S. +2 more
core +1 more source
Advanced Materials, EarlyView.Controlling the protein corona formation onto carbon nanomaterials (CNMs) enhances their functionalities as platforms for cancer theranostics. Here, we reviewed the effects of the intrinsic and acquired properties of CNMs on protein corona formation, the consequent biological and toxicological outcomes, and the strategies to reshape corona formation ...
Yajuan Zou +5 more
wiley +1 more source
Spontaneous Generation of Infectious Prion Disease in Transgenic Mice
Emerging Infectious Diseases, 2013 We generated transgenic mice expressing bovine cellular prion protein (PrPC) with a leucine substitution at codon 113 (113L). This protein is homologous to human protein with mutation 102L, and its genetic link with Gerstmann–Sträussler–Scheinker ...
Juan-María Torres +10 more
doaj +1 more source
A survey and a molecular dynamics study on the (central) hydrophobic region of prion proteins
, 2014 Prion diseases are invariably fatal neurodegenerative diseases that affect humans and animals. Unlike most other amyloid forming neurodegenerative diseases, these can be highly infectious. Prion diseases occur in a variety of species.
Wang, Feng, Zhang, Jiapu
core +1 more source
Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]
, 2010 Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Daggett, Valerie, van der Kamp, Marc W
core +2 more sources
Toxicity of novel C‐terminal prion protein fragments and peptides harbouring disease‐related C‐terminal mutations [PDF]
European Journal of Biochemistry, 2001 Mice expressing a C‐terminal fragment of the prion protein instead of wild‐type prion protein die from massive neuronal degeneration within weeks of birth. The C‐terminal region of PrPc (PrP121–231) expressed in these mice has an intrinsic neurotoxicity to cultured neurones.
M, Daniels, G M, Cereghetti, D R, Brown
openaire +2 more sources
Advanced Science, EarlyView.RNF213 is characterized as a dual‐functional antiviral effector. It directly mediates the degradation of the influenza A virus nucleoprotein (NP) while simultaneously activating the MDA5‐mediated innate immune signaling pathway. This coordinated response establishes a powerful host defense system against viral infection. ABSTRACT Influenza A virus (IAV)
Haoning Li +5 more
wiley +1 more source
Reflections on Cerebellar Neuropathology in Classical Scrapie
Biomolecules, 2021 In this review, the most important neuropathological changes found in the cerebella of sheep affected by classical natural scrapie are discussed. This disease is the oldest known of a group of unconventional “infections” caused by toxic prions of ...
Adolfo Toledano-Díaz +5 more
doaj +1 more source