Results 11 to 20 of about 7,097 (179)

PrPSc with Seeding Activity Extensively Overlaps with Proteinase-Resistant PrPSc Rather than Infectious PrPSc [PDF]

Pathogens, 2020
The disease-associated prion protein (PrPSc) has the ability to seed the conformational conversion of normal prion proteins into the amyloid fibril form.
Yoshifumi Iwamaru, Yuichi Matsuura, Kohtaro Miyazawa   +2 more
doaj   +3 more sources

The Structure of PrPSc Prions [PDF]

Pathogens, 2018
PrPSc (scrapie isoform of the prion protein) prions are the infectious agent behind diseases such as Creutzfeldt–Jakob disease in humans, bovine spongiform encephalopathy in cattle, chronic wasting disease in cervids (deer, elk, moose, and reindeer), as ...
Holger Wille, Jesús R. Requena
doaj   +3 more sources

Prnp Deletion Mitigates Muscle Fiber Type-Specific Sarcopenia Induced by Prion Infection in Mice. [PDF]

Immun Inflamm Dis
ABSTRACT Recent studies have shown that significant expression of PrPC protein is also present in skeletal muscle, and it plays a significant role in maintaining skeletal muscle homeostasis. Although the expression of PrPC in skeletal muscle has been clarified, the effects of PrPSc‐mediated prion protein infection on sarcopenia in mice and its ...
Liu W, Kim YC, Won SY, Kieu TTT, Kook SH, Jeong BH, Kim SH.   +6 more
europepmc   +2 more sources

Strain Traits of Intracranially Administered L-Type Bovine Spongiform Encephalopathy Prions Are not Significantly Modified During Intraspecies Transmission in Cynomolgus Monkeys. [PDF]

Microbiol Immunol
ABSTRACT Among the three prion strains of bovine spongiform encephalopathy (BSE), classical BSE (C‐BSE) prions are known causative agents of variant Creutzfeldt–Jakob disease. By contrast, human infections with L‐type (L‐) or H‐type (H‐) BSE prions have not been reported.
Hagiwara K, Shibata H, Tobiume M, Sato Y, Ohto K, Okabayashi S, Nakano N, Horiuchi M, Ono F.   +8 more
europepmc   +2 more sources

The Phenotypic Spectrum of Sporadic Creutzfeldt-Jakob Disease Cortical Subtype. [PDF]

Ann Neurol
Objective The objective of this study was to characterize the phenotypic spectrum of the rare sporadic Creutzfeldt‐Jakob disease cortical subtype (sCJDMM/MV2C) in a large multicentric autopsy cohort. Methods We evaluated clinical histories, biofluid markers, brain diffusion‐weighted (DW)‐magnetic resonance imaging (MRI), and electroencephalogram (EEG ...
Baiardi S, Vargiu CM, Appleby BS, Barria M, Bentivenga GM, Calì I, Carlà B, Cohen M, Giese A, Herms J, Kortelainen AM, Ladogana A, Mammana A, Ritchie D, Windl O, Capellari S, Parchi P.   +16 more
europepmc   +2 more sources

4-hydroxytamoxifen leads to PrPSc clearance by conveying both PrPC and PrPSc to lysosomes independently of autophagy [PDF]

Journal of Cell Science, 2013
Prion diseases are fatal neurodegenerative disorders involving the abnormal folding of a native cellular protein, named PrPC, to a malconformed aggregation-prone state, enriched in beta sheet secondary structure, denoted PrPSc. Recently, autophagy has garnered considerable attention as a cellular process with the potential to counteract ...
L. Marzo, Z. Marijanovic, D. Browman, Z. Chamoun, A. Caputo, ZURZOLO, CHIARA   +5 more
openaire   +4 more sources

PrPSc Prions: State of the Art [PDF]

, 2018
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of fatal and transmissible neurodegenerative disorders characterized by long incubation periods, misfolded prion protein (PrP) deposition, and usually spongiform vacuolation.
Jesús R. Requena (Ed.), Joaquín Castilla (Ed.)   +1 more
openaire   +3 more sources

Prion protein gene polymorphisms and accumulation of pathogenic prion protein (PrPSc) in a herd with previously confirmed scrapie cases

Bioscience Journal, 2015
Scrapie in sheep is associated with at least three polymorphisms in the prion protein gene (PRNP) on codons 136, 154, and 171. Countries where scrapie is endemic have been using breeding programs based on selection for the most resistant alleles.
Cristina Santos Sotomaior, David Driemeier, Juliano Leal, Rüdiger Daniel Ollhoff, Vanete Thomaz-Soccol   +4 more
doaj   +1 more source

Generation of human chronic wasting disease in transgenic mice

Acta Neuropathologica Communications, 2021
Chronic wasting disease (CWD) is a cervid prion disease caused by the accumulation of an infectious misfolded conformer (PrPSc) of cellular prion protein (PrPC). It has been spreading rapidly in North America and also found in Asia and Europe.
Zerui Wang, Kefeng Qin, Manuel V. Camacho, Ignazio Cali, Jue Yuan, Pingping Shen, Justin Greenlee, Qingzhong Kong, James A. Mastrianni, Wen-Quan Zou   +9 more
doaj   +1 more source

Strain-Dependent Effect of Macroautophagy on Abnormally Folded Prion Protein Degradation in Infected Neuronal Cells. [PDF]

PLoS ONE, 2015
Prion diseases are neurodegenerative disorders caused by the accumulation of abnormal prion protein (PrPSc) in the central nervous system. With the aim of elucidating the mechanism underlying the accumulation and degradation of PrPSc, we investigated the
Daisuke Ishibashi, Takujiro Homma, Takehiro Nakagaki, Takayuki Fuse, Kazunori Sano, Hanae Takatsuki, Ryuichiro Atarashi, Noriyuki Nishida   +7 more
doaj   +1 more source