Genetic prion disease–related mutation E196K displays a novel amyloid fibril structure revealed by cryo-EM

Prion protein glycans reduce intracerebral fibril formation and spongiosis in prion disease

(2020) Alejandro M. Sevillano et al.   JOURNAL OF CLINICAL INVESTIGATION

Novel tau filament fold in corticobasal degeneration

(2020) Wenjuan Zhang et al.   NATURE

Amyloid structure determination in RELION-3.1

(2020) Sjors H. W. Scheres   Acta Crystallographica Section D-Structural Biology

Cryo-EM structure of a human prion fibril with a hydrophobic, protease-resistant core

(2020) Calina Glynn et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Incomplete glycosylation during prion infection unmasks a prion protein epitope that facilitates prion detection and strain discrimination

(2020) Hae-Eun Kang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cryo-EM structure of an amyloid fibril formed by full-length human prion protein

(2020) Li-Qiang Wang et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

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

(2020) Emmanuel A. Asante et al.   PLOS BIOLOGY

Fibril structures of diabetes-related amylin variants reveal a basis for surface-templated assembly

(2020) Rodrigo Gallardo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Shifts and drifts in prion science

(2020) Adriano Aguzzi et al.   SCIENCE

Modeling PrPSc Generation Through Deformed Templating

(2020) Giovanni Spagnolli et al.   Frontiers in Bioengineering and Biotechnology

Structural features distinguishing infectious ex vivo mammalian prions from non-infectious fibrillar assemblies generated in vitro

(2019) Cassandra Terry et al.   Scientific Reports

Full atomistic model of prion structure and conversion

(2019) Giovanni Spagnolli et al.   PLoS Pathogens

Structures of fibrils formed by α-synuclein hereditary disease mutant H50Q reveal new polymorphs

(2019) David R. Boyer et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Prions, prionoids and protein misfolding disorders

(2018) Claudia Scheckel et al.   NATURE REVIEWS GENETICS

MotionCor2: anisotropic correction of beam-induced motion for improved cryo-electron microscopy

(2017) Shawn Q Zheng et al.   NATURE METHODS

Genetic PrP Prion Diseases

(2017) Mee-Ohk Kim et al.   Cold Spring Harbor Perspectives in Biology

Towards authentic transgenic mouse models of heritable PrP prion diseases

(2016) Joel C. Watts et al.   ACTA NEUROPATHOLOGICA

Ex vivomammalian prions are formed of paired double helical prion protein fibrils

(2016) Cassandra Terry et al.   Open Biology

The Structural Architecture of an Infectious Mammalian Prion Using Electron Cryomicroscopy

(2016) Ester Vázquez-Fernández et al.   PLoS Pathogens

Clinical findings and diagnosis in genetic prion diseases in Germany

(2015) Anna Krasnianski et al.   EUROPEAN JOURNAL OF EPIDEMIOLOGY

Pathological mutations H187R and E196K facilitate subdomain separation and prion protein conversion by destabilization of the native structure

(2015) San Hadži et al.   FASEB JOURNAL

CTFFIND4: Fast and accurate defocus estimation from electron micrographs

(2015) Alexis Rohou et al.   JOURNAL OF STRUCTURAL BIOLOGY

Structural Determinants of Phenotypic Diversity and Replication Rate of Human Prions

(2015) Jiri G. Safar et al.   PLoS Pathogens

Conformational Stability of Mammalian Prion Protein Amyloid Fibrils Is Dictated by a Packing Polymorphism within the Core Region

(2013) Nathan J. Cobb et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

High-resolution structure of infectious prion protein: the final frontier

(2012) Rodrigo Diaz-Espinoza et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

A Unifying Role for Prions in Neurodegenerative Diseases

(2012) S. B. Prusiner   SCIENCE

Biochemical Properties of Highly Neuroinvasive Prion Strains

(2012) Cyrus Bett et al.   PLoS Pathogens

Fibril Formation of the Rabbit/Human/Bovine Prion Proteins

(2011) Zheng Zhou et al.   BIOPHYSICAL JOURNAL

Common Structural Traits across Pathogenic Mutants of the Human Prion Protein and Their Implications for Familial Prion Diseases

(2011) Giulia Rossetti et al.   JOURNAL OF MOLECULAR BIOLOGY

Structural organization of brain-derived mammalian prions examined by hydrogen-deuterium exchange

(2011) Vytautas Smirnovas et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Genetic prion disease with codon 196 PRNP mutation: clinical and pathological findings

(2011) Gabi Schelzke et al.   NEUROBIOLOGY OF AGING

Features and development ofCoot

(2010) P. Emsley et al.   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

PHENIX: a comprehensive Python-based system for macromolecular structure solution

(2010) Paul D. Adams et al.   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

Electrostatics in the stability and misfolding of the prion protein: salt bridges, self energy, and solvationThis paper is one of a selection of papers published in this special issue entitled “Canadian Society of Biochemistry, Molecular & Cellular Biology 52nd Annual Meeting — Protein Folding: Principles and Diseases” and has undergone the Journal's usual peer review process.

(2010) Will C. Guest et al.   Biochemistry and Cell Biology

Salt bridges: Geometrically specific, designable interactions

(2010) Jason E. Donald et al.   PROTEINS-STRUCTURE FUNCTION AND BIOINFORMATICS

Prion hypothesis: the end of the controversy?

(2010) Claudio Soto   TRENDS IN BIOCHEMICAL SCIENCES

Low Micromolar Zinc Accelerates the Fibrillization of Human Tau via Bridging of Cys-291 and Cys-322

(2009) Zhong-Ying Mo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Natural and synthetic prion structure from X-ray fiber diffraction

(2009) H. Wille et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Initiation and elongation in fibrillation of ALS-linked superoxide dismutase

(2008) M. Chattopadhyay et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA