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2.7 Å cryo-EM structure of ex vivo RML prion fibrils

Nature communications, 2022-07, Vol.13 (1), p.4004-4004, Article 4004

2022

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A naturally occurring variant of the human prion protein completely prevents prion disease

Nature (London), 2015-06, Vol.522 (7557), p.478-481

2015

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Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS

PLoS biology, 2020-06, Vol.18 (6), p.e3000725-e3000725

2020

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A Novel Prion Disease Associated with Diarrhea and Autonomic Neuropathy

The New England journal of medicine, 2013-11, Vol.369 (20), p.1904-1914

2013

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Overexpression of mouse prion protein in transgenic mice causes a non-transmissible spongiform encephalopathy

Scientific reports, 2022-10, Vol.12 (1), p.17198-17198, Article 17198

2022

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Molecular pathology of human prion disease

Acta neuropathologica, 2011-01, Vol.121 (1), p.69-77

2011

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Human Prion Protein with Valine 129 Prevents Expression of Variant CJD Phenotype

Science (American Association for the Advancement of Science), 2004-12, Vol.306 (5702), p.1793-1796

2004

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Prion strains viewed through the lens of cryo-EM

Cell and tissue research, 2023-04, Vol.392 (1), p.167-178

2023

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Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin

Biochemical journal, 2008-12, Vol.416 (2), p.297-305

2008

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A structural basis for prion strain diversity

Nature chemical biology, 2023-05, Vol.19 (5), p.607

2023

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Isolation of proteinase K-sensitive prions using pronase E and phosphotungstic acid

PloS one, 2010-12, Vol.5 (12), p.e15679-e15679

2010

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Molecular classification of sporadic Creutzfeldt–Jakob disease

Brain (London, England : 1878), 2003-06, Vol.126 (6), p.1333-1346

2003

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Variant Creutzfeldt–Jakob Disease in a Patient with Heterozygosity at PRNP Codon 129

The New England journal of medicine, 2017-01, Vol.376 (3), p.292-294

2017

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Evaluation of plasma tau and neurofilament light chain biomarkers in a 12-year clinical cohort of human prion diseases

Molecular psychiatry, 2021-10, Vol.26 (10), p.5955-5966

2021

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Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins

Journal of general virology, 2009-03, Vol.90 (3), p.546-558

2009

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Recent Advances in Understanding Mammalian Prion Structure: A Mini Review

Frontiers in molecular neuroscience, 2019-07, Vol.12, p.169-169

2019

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Prion neuropathology follows the accumulation of alternate prion protein isoforms after infective titre has peaked

Nature communications, 2014-07, Vol.5 (1), p.4347, Article 4347

2014

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Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations

Brain (London, England : 1878), 2006-03, Vol.129 (3), p.676-685

2006

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Inherited prion disease with six octapeptide repeat insertional mutation- : molecular analysis of phenotypic heterogeneity

Brain (London, England : 1878), 2006-09, Vol.129 (Pt 9), p.2297-2317

2006

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Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein

Brain (London, England : 1878), 2006-06, Vol.129 (6), p.1557-1569

2006

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Kuru Prions and Sporadic Creutzfeldt-Jakob Disease Prions Have Equivalent Transmission Properties in Transgenic and Wild-Type Mice

Proceedings of the National Academy of Sciences - PNAS, 2008-03, Vol.105 (10), p.3885-3890

2008

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Spontaneous generation of mammalian prions

Proceedings of the National Academy of Sciences - PNAS, 2010-08, Vol.107 (32), p.14402-14406

2010

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Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report

The Lancet (British edition), 2006-12, Vol.368 (9552), p.2061-2067

2006

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Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein

Journal of general virology, 2010-10, Vol.91 (Pt 10), p.2651-2657

2010

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2.7 Å cryo-EM structure of ex vivo RML prion fibrils

A naturally occurring variant of the human prion protein completely prevents prion disease

Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS

A Novel Prion Disease Associated with Diarrhea and Autonomic Neuropathy

Overexpression of mouse prion protein in transgenic mice causes a non-transmissible spongiform encephalopathy

Molecular pathology of human prion disease

Human Prion Protein with Valine 129 Prevents Expression of Variant CJD Phenotype

Clinical Trial Simulations Based on Genetic Stratification and the Natural History of a Functional Outcome Measure in Creutzfeldt-Jakob Disease

Prion strains viewed through the lens of cryo-EM

Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin

A structural basis for prion strain diversity

Isolation of proteinase K-sensitive prions using pronase E and phosphotungstic acid

Molecular classification of sporadic Creutzfeldt–Jakob disease

Variant Creutzfeldt–Jakob Disease in a Patient with Heterozygosity at PRNP Codon 129

Evaluation of plasma tau and neurofilament light chain biomarkers in a 12-year clinical cohort of human prion diseases

Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins

Recent Advances in Understanding Mammalian Prion Structure: A Mini Review

Prion neuropathology follows the accumulation of alternate prion protein isoforms after infective titre has peaked

Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations

Inherited prion disease with six octapeptide repeat insertional mutation- : molecular analysis of phenotypic heterogeneity

Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein

Kuru Prions and Sporadic Creutzfeldt-Jakob Disease Prions Have Equivalent Transmission Properties in Transgenic and Wild-Type Mice

Spontaneous generation of mammalian prions

Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report

Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein

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