Results 11 to 20 of about 5,869 (184)

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

Prion protein amino acid sequence influences formation of authentic synthetic PrPSc

Scientific Reports, 2023
AbstractSynthetic prions, generated de novo from minimal, non-infectious components, cause bona fide prion disease in animals. Transmission of synthetic prions to hosts expressing syngeneic PrPC results in extended, variable incubation periods and incomplete attack rates.
Alyssa J. Block, Taylor C. York, Romilly Benedict, Jiyan Ma, Jason C. Bartz   +4 more
openaire   +3 more sources

Prion therapeutics: Lessons from the past

Prion, 2022
Prion diseases are a group of incurable zoonotic neurodegenerative diseases (NDDs) in humans and other animals caused by the prion proteins. The abnormal folding and aggregation of the soluble cellular prion proteins (PrPC) into scrapie isoform (PrPSc ...
Kyu Hwan Shim, Niti Sharma, Seong Soo A An   +2 more
doaj   +1 more source

Preclinical deposition of pathological prion protein PrPSc in muscles of hamsters orally exposed to scrapie [PDF]

Journal of Clinical Investigation, 2004
Recently, pathological prion protein PrP(Sc), the putative key constituent of infectious agents causing transmissible spongiform encephalopathies (TSEs), was found in muscles of rodents experimentally infected with scrapie and in patients with Creutzfeldt-Jakob disease (CJD).
Thomzig, A., Schulz-Schaeffer, Walter J., Kratzel, C., Mai, J., Beekes, M.   +4 more
openaire   +3 more sources

Characteristic distribution and molecular properties of normal cellular prion protein in human endocrine and exocrine tissues

Scientific Reports, 2022
Prion disease is an infectious and fatal neurodegenerative disease. Human prion disease autopsy studies have revealed abnormal prion protein (PrPSc) deposits in the central nervous system and systemic organs.
Sachiko Koyama, Hideko Noguchi, Kaoru Yagita, Hideomi Hamasaki, Masahiro Shijo, Motoi Yoshimura, Kohei Inoshita, Naokazu Sasagasako, Hiroyuki Honda   +8 more
doaj   +1 more source

PrPSc-like prion protein peptide inhibits the function of cellular prion protein [PDF]

Biochemical Journal, 2000
Mice lacking expression of the prion protein are protected against infection with prion disease. Neurodegeneration in prion disease requires the formation of the abnormal isoform of the prion protein (PrPSc) from host prion protein. Therefore expression of normal host prion protein is necessary for prion disease.
openaire   +2 more sources

Experimental Transmission of Abnormal Prion Protein (PrPsc) in the Small Intestinal Epithelial Cells of Neonatal Mice [PDF]

Veterinary Pathology, 2003
Using an immunohistochemical method, we attempted to detect the transmission of abnormal prion protein (PrPsc) to the enterocytes of the small intestine of neonatal mice by oral exposure with sheep brain affected by scrapie. Five 1-day-old neonatal mice were exposed by oral inoculation to the homogenized brain of a scrapie-affected sheep. In the small
M, Okamoto, H, Furuoka, M, Horiuchi, T, Noguchi, K, Hagiwara, Y, Muramatsu, K, Tomonaga, M, Tsuji, C, Ishihara, K, Ikuta, H, Taniyama   +10 more
openaire   +2 more sources

Exploration of the main sites for the transformation of normal prion protein (PrPC) into pathogenic prion protein (PrPsc)

Journal of Veterinary Research, 2017
Introduction: The functions and mechanisms of prion proteins (PrPC) are currently unknown, but most experts believe that deformed or pathogenic prion proteins (PrPSc) originate from PrPC, and that there may be plural main sites for the conversion of ...
Liu Xi-Lin, Feng Xiao-Li, Wang Guang-Ming, Gong Bin-Bin, Ahmad Waqas, Liu Nan-Nan, Zhang Yuan-Yuan, Yang Li, Ren Hong-Lin, Cui Shu-Sen   +9 more
doaj   +1 more source

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

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   +1 more source