Probing Early Misfolding Events in Prion Protein Mutants by NMR Spectroscopy

Report about Four Novel Mutations in the Prion Protein Gene

(2013) Gabi Schelzke et al.   DEMENTIA AND GERIATRIC COGNITIVE DISORDERS

Cellular Form of Prion Protein Inhibits Reelin-Mediated Shedding of Caspr from the Neuronal Cell Surface to Potentiate Caspr-Mediated Inhibition of Neurite Outgrowth

(2013) V. Devanathan et al.   JOURNAL OF NEUROSCIENCE

PrPC Controls via Protein Kinase A the Direction of Synaptic Plasticity in the Immature Hippocampus

(2013) M. D. Caiati et al.   JOURNAL OF NEUROSCIENCE

Zinc Drives a Tertiary Fold in the Prion Protein with Familial Disease Mutation Sites at the Interface

(2013) Ann R. Spevacek et al.   STRUCTURE

Structural basis for the protective effect of the human prion protein carrying the dominant-negative E219K polymorphism

(2012) Ivana Biljan et al.   BIOCHEMICAL JOURNAL

Effects of the Pathological Q212P Mutation on Human Prion Protein Non-Octarepeat Copper-Binding Site

(2012) Paola D’Angelo et al.   BIOCHEMISTRY

Structural Rearrangements at Physiological pH: Nuclear Magnetic Resonance Insights from the V210I Human Prion Protein Mutant

(2012) Ivana Biljan et al.   BIOCHEMISTRY

The N-Terminal, Polybasic Region of PrPC Dictates the Efficiency of Prion Propagation by Binding to PrPSc

(2012) J. A. Turnbaugh et al.   JOURNAL OF NEUROSCIENCE

Isolation of Novel Synthetic Prion Strains by Amplification in Transgenic Mice Coexpressing Wild-Type and Anchorless Prion Proteins

(2012) G. J. Raymond et al.   JOURNAL OF VIROLOGY

Prion propagation, toxicity and degradation

(2012) Adriano Aguzzi et al.   NATURE NEUROSCIENCE

Mutant PrP Suppresses Glutamatergic Neurotransmission in Cerebellar Granule Neurons by Impairing Membrane Delivery of VGCC α2δ-1 Subunit

(2012) Assunta Senatore et al.   NEURON

Review: Creutzfeldt-Jakob disease: prion protein type, disease phenotype and agent strain

(2012) M. W. Head et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Non-amyloid and amyloid prion protein deposits in prion-infected mice differ in blockage of interstitial brain fluid

(2012) A. Rangel et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Mapping the Prion Protein Distribution in Marsupials: Insights from Comparing Opossum with Mouse CNS

(2012) Ilaria Poggiolini et al.   PLoS One

Disruption of the X-loop turn of the prion protein linked to scrapie resistance

(2012) A. D. Scouras et al.   PROTEIN ENGINEERING DESIGN & SELECTION

Propagation of RML Prions in Mice Expressing PrP Devoid of GPI Anchor Leads to Formation of a Novel, Stable Prion Strain

(2012) Sukhvir Paul Mahal et al.   PLoS Pathogens

Mutation directional selection sheds light on prion pathogenesis

(2011) Liang Shen et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Copper Alters Aggregation Behavior of Prion Protein and Induces Novel Interactions between Its N- and C-terminal Regions

(2011) Abhay Kumar Thakur et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Toward the Molecular Basis of Inherited Prion Diseases: NMR Structure of the Human Prion Protein with V210I Mutation

(2011) Ivana Biljan et al.   JOURNAL OF MOLECULAR BIOLOGY

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

Dynamic Diagnosis of Familial Prion Diseases Supports the β2-α2 Loop as a Universal Interference Target

(2011) Massimiliano Meli et al.   PLoS One

Spontaneous generation of anchorless prions in transgenic mice

(2011) J. Stohr et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An overview of animal prion diseases

(2011) Muhammad Imran et al.   Virology Journal

Prions

(2011) D. W. Colby et al.   Cold Spring Harbor Perspectives in Biology

Human PrP90-231-induced cell death is associated with intracellular accumulation of insoluble and protease-resistant macroaggregates and lysosomal dysfunction

(2011) S Thellung et al.   Cell Death & Disease

Heterozygosity at Polymorphic Codon 219 in Variant Creutzfeldt-Jakob Disease

(2010) Ana Lukic et al.   ARCHIVES OF NEUROLOGY

Diverse Effects on the Native β-Sheet of the Human Prion Protein Due to Disease-Associated Mutations

(2010) Wei Chen et al.   BIOCHEMISTRY

Influence of pH on the Human Prion Protein: Insights into the Early Steps of Misfolding

(2010) Marc W. van der Kamp et al.   BIOPHYSICAL JOURNAL

Prospective 10-year surveillance of human prion diseases in Japan

(2010) I. Nozaki et al.   BRAIN

Prion Protein Amyloid Formation Involves Structural Rearrangements in the C-Terminal Domain

(2010) Jitendra Kumar et al.   CHEMBIOCHEM

PRNP allelic series from 19 years of prion protein gene sequencing at the MRC Prion Unit

(2010) Jon A. Beck et al.   HUMAN MUTATION

High hydrophobic amino acid exposure is responsible of the neurotoxic effects induced by E200K or D202N disease-related mutations of the human prion protein

(2010) Alessandro Corsaro et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Unique Structural Characteristics of the Rabbit Prion Protein

(2010) Yi Wen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prion Fibrillization Is Mediated by a Native Structural Element That Comprises Helices H2 and H3

(2010) Miquel Adrover et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Neurodevelopmental expression and localization of the cellular prion protein in the central nervous system of the mouse

(2010) Stefano Benvegnù et al.   JOURNAL OF COMPARATIVE NEUROLOGY

The Unfolded State of the Murine Prion Protein and Properties of Single-point Mutants Related to Human Prion Diseases

(2010) Christian Gerum et al.   JOURNAL OF MOLECULAR BIOLOGY

Pathogenic Mutations in the Hydrophobic Core of the Human Prion Protein Can Promote Structural Instability and Misfolding

(2010) Marc W. van der Kamp et al.   JOURNAL OF MOLECULAR BIOLOGY

Horse Prion Protein NMR Structure and Comparisons with Related Variants of the Mouse Prion Protein

(2010) Daniel R. Pérez et al.   JOURNAL OF MOLECULAR BIOLOGY

NMR Structure of the Human Prion Protein with the Pathological Q212P Mutation Reveals Unique Structural Features

(2010) Gregor Ilc et al.   PLoS One

Prion neurodegeneration

(2010) Giovanna R. Mallucci   Prion

Prion protein interaction with stress-inducible protein 1 enhances neuronal protein synthesis via mTOR

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

Structural facets of disease-linked human prion protein mutants: A molecular dynamic study

(2010) Giulia Rossetti et al.   PROTEINS-STRUCTURE FUNCTION AND BIOINFORMATICS

Prion protein amyloidosis with divergent phenotype associated with two novel nonsense mutations in PRNP

(2009) Casper Jansen et al.   ACTA NEUROPATHOLOGICA

Genetics and molecular pathogenesis of sporadic and hereditary cerebral amyloid angiopathies

(2009) Tamas Revesz et al.   ACTA NEUROPATHOLOGICA

Unfolded-State Structure and Dynamics Influence the Fibril Formation of Human Prion Protein

(2009) Christian Gerum et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

Prion Proteins with Pathogenic and Protective Mutations Show Similar Structure and Dynamics

(2009) Sung-Hun Bae et al.   BIOCHEMISTRY

PRNP variation in UK sporadic and variant Creutzfeldt Jakob disease highlights genetic risk factors and a novel non-synonymous polymorphism

(2009) Matthew T Bishop et al.   BMC Medical Genetics

Prion Protein NMR Structure from Tammar Wallaby (Macropus eugenii) Shows that the β2–α2 Loop Is Modulated by Long-Range Sequence Effects

(2009) Barbara Christen et al.   JOURNAL OF MOLECULAR BIOLOGY

Unique clinicopathological features and PrP profiles in the first autopsied case of dura mater graft-associated Creutzfeldt–Jakob disease with codon 219 lysine allele observed in Japanese population

(2009) Masamichi Ikawa et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Sensors and regulators of intracellular pH

(2009) Joseph R. Casey et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure

(2009) Simon Mead et al.   NEW ENGLAND JOURNAL OF MEDICINE

Regulation of GABAA and Glutamate Receptor Expression, Synaptic Facilitation and Long-Term Potentiation in the Hippocampus of Prion Mutant Mice

(2009) Alejandra Rangel et al.   PLoS One

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

Prion protein biosynthesis and its emerging role in neurodegeneration

(2009) Oishee Chakrabarti et al.   TRENDS IN BIOCHEMICAL SCIENCES

Trans-Dominant Inhibition of Prion Propagation In Vitro Is Not Mediated by an Accessory Cofactor

(2009) James C. Geoghegan et al.   PLoS Pathogens

Identification of an Intracellular Site of Prion Conversion

(2009) Zrinka Marijanovic et al.   PLoS Pathogens

Selective Processing and Metabolism of Disease-Causing Mutant Prion Proteins

(2009) Aarthi Ashok et al.   PLoS Pathogens

Cerebral amyloid angiopathy with co-localization of prion protein and beta-amyloid in an 85-year-old patient with sporadic Creutzfeldt–Jakob disease

(2008) Claire Paquet et al.   ACTA NEUROPATHOLOGICA

The Prion's Elusive Reason for Being

(2008) Adriano Aguzzi et al.   Annual Review of Neuroscience

Copper and the structural biology of the prion protein

(2008) John H. Viles et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Human Prion Protein (PrP) 219K Is Converted to PrPScbut Shows Heterozygous Inhibition in Variant Creutzfeldt-Jakob Disease Infection

(2008) Masaki Hizume et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prion protein attenuates excitotoxicity by inhibiting NMDA receptors

(2008) Houman Khosravani et al.   JOURNAL OF CELL BIOLOGY

NMR Structure of the Bank Vole Prion Protein at 20 °C Contains a Structured Loop of Residues 165–171

(2008) Barbara Christen et al.   JOURNAL OF MOLECULAR BIOLOGY

Cryo-Immunogold Electron Microscopy for Prions: Toward Identification of a Conversion Site

(2008) S. F. Godsave et al.   JOURNAL OF NEUROSCIENCE

Physiology of the Prion Protein

(2008) Rafael Linden et al.   PHYSIOLOGICAL REVIEWS