Prions can exist as different strains that consist of conformational variants of the misfolded, pathogenic prion protein isoform PrPSc. Defined by stably transmissible biological and biochemical properties, strains have been identified in a spectrum of ...
Sheng Chun Chang +7 more
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Recent Advances in Cell-Free PrPSc Amplification Technique
The development of amplification technology for abnormal forms of prion protein in vitro has had a great impact on the field of prion research. This novel technology has generated new possibilities for understanding the molecular basis of prions and for developing an early diagnostic test for prion diseases.
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Elucidation of the structure of PrPSc continues to be one major challenge in prion research. Molecular basis of the biology of prion protein, such as the molecular mechanism of prion replication and aggregation, the species barrier and the pathogenesis of neurodegeneration will not be understood until the structure is solved. Given that high-resolution
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Quinacrine Repurposing for PrPSc Aggregation Inhibition
Quinacrine binds directly to the cellular prion protein (PrPC) and inhibits its conversion to the pathogenic scrapie form (PrPSc) by stabilizing the native α-helical structure. The drug interferes with the β-sheet formation characteristic of misfolded prions and may enhance clearance of existing aggregates through autophagy activation.
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Generation of Infectious Prions Amenable to Site-Specific Click Chemistry. [PDF]
Campbell RG +3 more
europepmc +1 more source
Chemo-omic pipeline enables discovery of prion synaptotoxic pathways and inhibitory drugs. [PDF]
Le NTT +9 more
europepmc +1 more source
Incomplete protection of the K<sub>222</sub> polymorphism of the prion protein gene after intracerebral exposure to caprine BSE in goats: preliminary follow-up in S<sub>127</sub> carriers. [PDF]
Sola D +12 more
europepmc +1 more source
Limited transmission of cervid prions to nonhuman primates provides insights into the zoonotic potential of chronic wasting disease. [PDF]
Hannaoui S +16 more
europepmc +1 more source
Strain-specific propagation of variant Creutzfeldt-Jakob disease prions in humanized neural cells. [PDF]
Rayner MLD +13 more
europepmc +1 more source
Distinct prion conformers from brain and peripheral tissues of gene-targeted mice produce convergent CWD strain properties. [PDF]
DeFranco JP +7 more
europepmc +1 more source

