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61
Burrowing into prion disease.
Published 2001“…Burrowing is a simple, sensitive, objective, ethological measure, sensitive to preclinical prion disease. Other potential applications are in transgenic and knockout mice, models of ageing and Alzheimer's disease, and pharmacology, particularly neuroleptics.…”
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62
Glycosylation and prion protein.
Published 2002“…Recent advances have elucidated the detailed glycosylation of the prion protein and highlighted the size of the sugars, which shield large areas of the protein and confer some conformational stability on the normal cellular form. …”
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63
There is no safe dose of prions.
Published 2011“…These data are compared to results from a within-host model in which prions accumulate according to a stochastic birth-death process. …”
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64
Thermodynamic Stabilization of the Folded Domain of Prion Protein Inhibits Prion Infection in Vivo
Published 2013-07-01“…Prion diseases, or transmissible spongiform encephalopathies (TSEs), are associated with the conformational conversion of the cellular prion protein, PrPC, into a protease-resistant form, PrPSc. …”
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65
Membrane Domain Localization and Interaction of the Prion-Family Proteins, Prion and Shadoo with Calnexin
Published 2021-12-01“…The cellular prion protein (PrP<sup>C</sup>) is renowned for its infectious conformational isoform PrP<sup>Sc</sup>, capable of templating subsequent conversions of healthy PrP<sup>C</sup>s and thus triggering the group of incurable diseases known as transmissible spongiform encephalopathies. …”
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66
Accelerated high fidelity prion amplification within and across prion species barriers.
Published 2008-08-01“…Experimental obstacles have impeded our ability to study prion transmission within and, more particularly, between species. …”
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67
Prion and Prion-Like Protein Strains: Deciphering the Molecular Basis of Heterogeneity in Neurodegeneration
Published 2019-03-01Subjects: “…prion…”
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68
Failure To Detect Prion Infectivity in Ticks following Prion-Infected Blood Meal
Published 2020-10-01Subjects: Get full text
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69
Radotinib Decreases Prion Propagation and Prolongs Survival Times in Models of Prion Disease
Published 2023-07-01Subjects: Get full text
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70
Correction: Glycoform-Selective Prion Formation in Sporadic and Familial Forms of Prion Disease.
Published 2013-01-01Get full text
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71
Contrasting Effects of Two Lipid Cofactors of Prion Replication on the Conformation of the Prion Protein.
Published 2015-01-01“…Recent studies introduced two experimental protocols for converting full-length recombinant prion protein (rPrP) purified from E.coli into the infectious prion state (PrPSc) with high infectivity titers. …”
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72
Correction: Glycoform-Selective Prion Formation in Sporadic and Familial Forms of Prion Disease
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73
Evidence for preexisting prion substrain diversity in a biologically cloned prion strain.
Published 2023-09-01“…Prions are composed of PrPSc, the disease specific conformation of the host encoded prion protein. …”
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74
The Way forward for the Origin of Life: Prions and Prion-Like Molecules First Hypothesis
Published 2021-08-01Subjects: “…prions…”
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75
Ex vivomammalian prions are formed of paired double helical prion protein fibrils
Published 2016“…Mammalian prions are hypothesized to be fibrillar or amyloid forms of prion protein (PrP), but structures observed to date have not been definitively correlated with infectivity and the three-dimensional structure of infectious prions has remained obscure. …”
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76
The way forward for the origin of life: prions and prion-like molecules first hypothesis
Published 2021“…In this paper the hypothesis that prions and prion-like molecules could have initiated the chemical evolutionary process which led to the eventual emergence of life is reappraised. …”
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77
Bank vole prion protein extends the use of RT-QuIC assays to detect prions in a range of inherited prion diseases
Published 2021-03-01“…Abstract The cerebrospinal fluid (CSF) real-time quaking-induced conversion assay (RT-QuIC) is an ultrasensitive prion amyloid seeding assay for diagnosis of sporadic Creutzfeldt–Jakob disease (CJD) but several prion strains remain unexplored or resistant to conversion with commonly used recombinant prion protein (rPrP) substrates. …”
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78
Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein.
Published 2013-01-01“…Prions are infectious agents causing fatal neurodegenerative diseases of humans and animals. …”
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79
<i>C. elegans</i> Models to Study the Propagation of Prions and Prion-Like Proteins
Published 2020-08-01Subjects: “…prions…”
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80
A unifying model for the propagation of prion proteins in yeast brings insight into the [PSI+] prion.
Published 2020-05-01“…The use of yeast systems to study the propagation of prions and amyloids has emerged as a crucial aspect of the global endeavor to understand those mechanisms. …”
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