Results 11 to 20 of about 1,549,882 (213)

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   +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   +2 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

Proteostasis unbalance in prion diseases: Mechanisms of neurodegeneration and therapeutic targets

Frontiers in Neuroscience, 2022
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are progressive neurodegenerative disorders of the central nervous system that affect humans and animals as sporadic, inherited, and infectious forms.
Stefano Thellung, Alessandro Corsaro, Irene Dellacasagrande, Mario Nizzari, Martina Zambito, Tullio Florio, Tullio Florio   +6 more
doaj   +1 more source

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification [PDF]

, 2009
Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrPSc, and the methionine (M)/valine (V) polymorphic codon 129 of the prion ...
Maurizio Pocchiari, Roncaroli F, Cras, Patrick, Patrich Cras, Anna Ladogana, Giese, Armin, Cras, Patrich, Pocchiari M, Ladogana, Anna, Federico Roncaroli, Ladogana A, Bernardino Ghetti, Jan P. M. Langeveld, Strammiello, R., Zerr, I., Ghetti, B., Ghetti B, Roncaroli, Federico, Pocchiari, Maurizio, Langeveld, Jan P.M., Inga Zerr, Cras, P., Notari, Silvio, Cras P, Giese, A., Roncaroli, F., Parchi, Piero, Notari, S., Kretzschmar H, Langeveld JP, Rosaria Strammiello, Ghetti, Bernardino, Zerr, Inga, Kretzschmar, Hans A., Armin Giese, Parchi, P., Capellari, S., Hans Kretzschmar, Notari S, Pocchiari, M., Kretzschmar, Hans, Ladogana, A., Giese A, Langeveld, J.P.M., Capellari, Sabina, Strammiello, Rosaria, Piero Parchi, Silvio Notari, Zerr I, Langeveld, Jan P. M., Kretzschmar, H., Sabina Capellari   +51 more
core   +1 more source

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

Trapping Prion Protein in the Endoplasmic Reticulum Impairs PrPC Maturation and Prevents PrPSc Accumulation [PDF]

Journal of Biological Chemistry, 2005
The conversion of the normal cellular prion protein (PrP(C)) into the abnormal scrapie isoform (PrP(Sc)) is a key feature of prion diseases. The pathogenic mechanisms and the subcellular sites of the conversion are complex and not completely understood.
Cardinale, A, Filesi, I, Vetrugno, V, Pocchiari, M, Sy, MS, BIOCCA, SILVIA   +5 more
openaire   +4 more sources

A new method for the Characterization of Strain-Specific Conformational Stability of Protease-Sensitive and Protease Resistant PrPSc [PDF]

, 2010
Although proteinacious in nature, prions exist as strains with specific self-perpetuating biological properties. Prion strains are thought to be associated with different conformers of PrPSc, a disease-associated isoform of the host-encoded cellular ...
Gabriele Vaccari, Laura Pirisinu, Marcon Stefano, Fazzi, P., Pirisinu Laura, Michele Di Bari, Nonno Romolo, Bari, M., Di, Marcon, S., Langeveld Jan, Paola Fazzi, Cardone, F., Vaccari Gabriele, Vaccari, G., Langeveld, J.P.M., Galeno Roberta, Roberta Galeno, Pirisinu, L., Esposito Elena, Fazzi Paola, Jan Langeveld, Nonno, R., Umberto Agrimi, D'Agostino, C., Elena Esposito, Stefano Marcon, Esposito, E., Claudia D'Agostino, Agrimi Umberto, Agrimi, U., Agostino, C., D', Di Bari, M., D'Agostino Claudia, Romolo Nonno, Di Bari Michele   +34 more
core   +2 more sources

Synthetic prions generated in vitro are similar to a newly identified subpopulation of PrPSc from sporadic Creutzfeldt-Jakob disease [PDF]

, 2005
In recent studies, the amyloid form of recombinant prion protein (PrP) encompassing residues 89-230 (rPrP 89-230) produced in vitro induced transmissible prion disease in mice.
Bocharova, O V, Salnikov, V V, Gill, A C, Vadim V. Salnikov, Ilia V. Baskakov, Leonid Breydo, Breydo, L, Olga V. Bocharova, Andrew C. Gill, Baskakov, I V   +9 more
core   +1 more source

PrPSc complexity in different forms of Creutzfeldt-Jakob disease identified using biochemical approaches [PDF]

, 2010
Transmissible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative diseases affecting humans and animal species.
Choi, Young Pyo
core   +3 more sources