Human prion diseases: Molecular, cellular and population biology

Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA

(2012) Piero Parchi et al.   ACTA NEUROPATHOLOGICA

MM2-Thalamic Creutzfeldt-Jakob Disease: Neuropathological, Biochemical and Transmission Studies Identify a Distinctive Prion Strain

(2012) Fabio Moda et al.   BRAIN PATHOLOGY

The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease

(2012) Oliver D. King et al.   BRAIN RESEARCH

Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein

(2012) R. Wilson et al.   JOURNAL OF GENERAL VIROLOGY

Prions are a common mechanism for phenotypic inheritance in wild yeasts

(2012) Randal Halfmann et al.   NATURE

Highly neurotoxic monomeric  -helical prion protein

(2012) M. Zhou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cofactor molecules maintain infectious conformation and restrict strain properties in purified prions

(2012) N. R. Deleault et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Risk of Prion Zoonoses

(2012) J. Collinge   SCIENCE

Facilitated Cross-Species Transmission of Prions in Extraneural Tissue

(2012) V. Beringue et al.   SCIENCE

A Unifying Role for Prions in Neurodegenerative Diseases

(2012) S. B. Prusiner   SCIENCE

Highly Efficient Prion Transmission by Blood Transfusion

(2012) Olivier Andréoletti et al.   PLoS Pathogens

Co-Occurrence of Types 1 and 2 PrP res in Sporadic Creutzfeldt-Jakob Disease MM1

(2011) Atsushi Kobayashi et al.   AMERICAN JOURNAL OF PATHOLOGY

Pathogenic protein seeding in alzheimer disease and other neurodegenerative disorders

(2011) Mathias Jucker et al.   ANNALS OF NEUROLOGY

The Seeds of Neurodegeneration: Prion-like Spreading in ALS

(2011) Magdalini Polymenidou et al.   CELL

Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry

(2011) Pelagia Deriziotis et al.   EMBO JOURNAL

Biochemical and strain properties of CJD prions: complexity versus simplicity

(2011) Stéphane Haïk et al.   JOURNAL OF NEUROCHEMISTRY

Genetic Informational RNA Is Not Required for Recombinant Prion Infectivity

(2011) F. Wang et al.   JOURNAL OF VIROLOGY

De novo induction of amyloid-β deposition in vivo

(2011) R Morales et al.   MOLECULAR PSYCHIATRY

Prion propagation and toxicity in vivo occur in two distinct mechanistic phases

(2011) Malin K. Sandberg et al.   NATURE

De novo generation of prion strains

(2011) David W. Colby et al.   NATURE REVIEWS MICROBIOLOGY

All Clinically-Relevant Blood Components Transmit Prion Disease following a Single Blood Transfusion: A Sheep Model of vCJD

(2011) Sandra McCutcheon et al.   PLoS One

Protein-only mechanism induces self-perpetuating changes in the activity of neuronal Aplysia cytoplasmic polyadenylation element binding protein (CPEB)

(2011) S. U. Heinrich et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells

(2011) Christian Münch et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Sheep and Goat BSE Propagate More Efficiently than Cattle BSE in Human PrP Transgenic Mice

(2011) Danielle Padilla et al.   PLoS Pathogens

Variably protease-sensitive prionopathy in a PRNP codon 129 heterozygous UK patient with co-existing tau, α synuclein and Aβ pathology

(2010) Mark W. Head et al.   ACTA NEUROPATHOLOGICA

Molecular biology and pathology of prion strains in sporadic human prion diseases

(2010) Pierluigi Gambetti et al.   ACTA NEUROPATHOLOGICA

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

(2010) Wen-Quan Zou et al.   ANNALS OF NEUROLOGY

Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease

(2010) P. Parchi et al.   BRAIN

Correlation of Polydispersed Prion Protein and Characteristic Pathology in the Thalamus in Variant Creutzfeldt-Jakob Disease: Implication of Small Oligomeric Species

(2010) Young Pyo Choi et al.   BRAIN PATHOLOGY

Aplysia CPEB Can Form Prion-like Multimers in Sensory Neurons that Contribute to Long-Term Facilitation

(2010) Kausik Si et al.   CELL

Prion amyloid structure explains templating: how proteins can be genes

(2010) Reed B. Wickner et al.   FEMS YEAST RESEARCH

The genetics of prion diseases

(2010) James A Mastrianni   GENETICS IN MEDICINE

Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia

(2010) A. PEDEN et al.   HAEMOPHILIA

Prion-like disorders: blurring the divide between transmissibility and infectivity

(2010) M. Cushman et al.   JOURNAL OF CELL SCIENCE

Comparison of the level, distribution and form of disease-associated prion protein in variant and sporadic Creutzfeldt-Jakob diseased brain using conformation-dependent immunoassay and Western blot

(2010) Y. P. Choi et al.   JOURNAL OF GENERAL VIROLOGY

Human embryonic stem cells rapidly take up and then clear exogenous human and animal prions in vitro

(2010) Zuzana Krejciova et al.   JOURNAL OF PATHOLOGY

Experimental Verification of a Traceback Phenomenon in Prion Infection

(2010) A. Kobayashi et al.   JOURNAL OF VIROLOGY

Distinct Stability States of Disease-Associated Human Prion Protein Identified by Conformation-Dependent Immunoassay

(2010) Y. P. Choi et al.   JOURNAL OF VIROLOGY

Increased Susceptibility of Human-PrP Transgenic Mice to Bovine Spongiform Encephalopathy Infection following Passage in Sheep

(2010) C. Plinston et al.   JOURNAL OF VIROLOGY

Uncertainty in the Tail of the Variant Creutzfeldt-Jakob Disease Epidemic in the UK

(2010) Tini Garske et al.   PLoS One

Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties

(2010) M. T. Bishop et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Darwinian Evolution of Prions in Cell Culture

(2010) J. Li et al.   SCIENCE

Epigenetics in the Extreme: Prions and the Inheritance of Environmentally Acquired Traits

(2010) Randal Halfmann et al.   SCIENCE

Prion-like aggregates: infectious agents in human disease

(2010) Gunilla T. Westermark et al.   TRENDS IN MOLECULAR MEDICINE

The propagation of prion-like protein inclusions in neurodegenerative diseases

(2010) Michel Goedert et al.   TRENDS IN NEUROSCIENCES

The Physical Relationship between Infectivity and Prion Protein Aggregates Is Strain-Dependent

(2010) Philippe Tixador et al.   PLoS Pathogens

Protease-Sensitive Synthetic Prions

(2010) David W. Colby et al.   PLoS Pathogens

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

(2009) Piero Parchi et al.   ACTA NEUROPATHOLOGICA

Further characterisation of the prion protein molecular types detectable in the NIBSC Creutzfeldt–Jakob disease brain reference materials

(2009) Helen M. Yull et al.   BIOLOGICALS

Sporadic Creutzfeldt-Jakob disease: discrete subtypes or a spectrum of disease?

(2009) M. W. Head et al.   BRAIN

Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt–Jakob disease: its effect on the phenotype and prion-type characteristics

(2009) Ignazio Cali et al.   BRAIN

Three serial passages of bovine spongiform encephalopathy in sheep do not significantly affect discriminatory test results

(2009) M. Stack et al.   JOURNAL OF GENERAL VIROLOGY

Development of oligomeric prion-protein aggregates in a mouse model of prion disease

(2009) Kensuke Sasaki et al.   JOURNAL OF PATHOLOGY

Variant CJD in an individual heterozygous for PRNP codon 129

(2009) Diego Kaski et al.   LANCET

The Transcellular Spread of Cytosolic Amyloids, Prions, and Prionoids

(2009) Adriano Aguzzi et al.   NEURON

Prion protein oligomers in Creutzfeldt-Jakob disease detected by gel-filtration centrifuge columns

(2009) Haruhiko Minaki et al.   NEUROPATHOLOGY

Pathological investigation of the first blood donor and recipient pair linked by transfusion-associated variant Creutzfeldt-Jakob disease transmission

(2009)   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

A case of protease sensitive prionopathy in a patient in the UK

(2009)   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Prion Strain Discrimination Based on Rapid In Vivo Amplification and Analysis by the Cell Panel Assay

(2009) Yervand Eduard Karapetyan et al.   PLoS One

Inclusion formation and neuronal cell death through neuron-to-neuron transmission of  -synuclein

(2009) P. Desplats et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Distinct conformations of in vitro and in vivo amyloids of huntingtin-exon1 show different cytotoxicity

(2009) Y. Nekooki-Machida et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Design and construction of diverse mammalian prion strains

(2009) D. W. Colby et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A novel human disease with abnormal prion protein sensitive to protease

(2008) Pierluigi Gambetti et al.   ANNALS OF NEUROLOGY

Crossing the Species Barrier by PrPSc Replication In Vitro Generates Unique Infectious Prions

(2008) Joaquín Castilla et al.   CELL

Cell-free propagation of prion strains

(2008) Joaquín Castilla et al.   EMBO JOURNAL

Conformational Diversity of Wild-type Tau Fibrils Specified by Templated Conformation Change

(2008) Bess Frost et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assay

(2008) Michael Jones et al.   NEUROREPORT

Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice

(2008) J. D. F. Wadsworth et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA