Prion Protein Biology Through the Lens of Liquid-Liquid Phase Separation

FUS and TDP-43 Phases in Health and Disease

(2021) Bede Portz et al.   TRENDS IN BIOCHEMICAL SCIENCES

The interplay between a GC-rich oligonucleotide and copper ions on prion protein conformational and phase transitions

(2021) Yulli M. Passos et al.   INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES

Biomolecular condensates at the nexus of cellular stress, protein aggregation disease and ageing

(2021) Simon Alberti et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mechanism of misfolding of the human prion protein revealed by a pathological mutation

(2021) Máximo Sanz-Hernández et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Liquid–liquid phase separation of tau: From molecular biophysics to physiology and disease

(2021) Sandeep K. Rai et al.   PROTEIN SCIENCE

The N-terminal domain of the prion protein is required and sufficient for liquid–liquid phase separation: A crucial role of the Aβ-binding domain

(2021) Janine Kamps et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prevalence and species distribution of the low-complexity, amyloid-like, reversible, kinked segment (LARKS) structural motif in amyloid-like fibrils

(2021) Michael P. Hughes et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Generic nature of the condensed states of proteins

(2021) Monika Fuxreiter et al.   NATURE CELL BIOLOGY

The Cellular Prion Protein Increases the Uptake and Toxicity of TDP-43 Fibrils

(2021) Carlo Scialò et al.   Viruses-Basel

Catalytic coacervate crucibles

(2021) Samrat Mukhopadhyay   Nature Chemistry

An intrinsically disordered pathological prion variant Y145Stop converts into self-seeding amyloids via liquid–liquid phase separation

(2021) Aishwarya Agarwal et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Prion protein PrP nucleic acid binding and mobilization implicates retroelements as the replicative component of transmissible spongiform encephalopathy

(2020) Richard Lathe et al.   ARCHIVES OF VIROLOGY

Valence and patterning of aromatic residues determine the phase behavior of prion-like domains

(2020) Erik W. Martin et al.   SCIENCE

Biomolecular Phase Separation: From Molecular Driving Forces to Macroscopic Properties

(2020) Gregory L. Dignon et al.   Annual Review of Physical Chemistry

Liquid–Liquid Phase Separation and Its Mechanistic Role in Pathological Protein Aggregation

(2020) W. Michael Babinchak et al.   JOURNAL OF MOLECULAR BIOLOGY

The uptake of tau amyloid fibrils is facilitated by the cellular prion protein and hampers prion propagation in cultured cells

(2020) Elena De Cecco et al.   JOURNAL OF NEUROCHEMISTRY

Phase Separation—A Physical Mechanism for Organizing Information and Biochemical Reactions

(2020) Rohit V. Pappu   DEVELOPMENTAL CELL

Biochemical Timekeeping Via Reentrant Phase Transitions

(2020) Bede Portz et al.   JOURNAL OF MOLECULAR BIOLOGY

Widespread occurrence of the droplet state of proteins in the human proteome

(2020) Maarten Hardenberg et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Copper Binding Regulates Cellular Prion Protein Function

(2019) Xuan T. A. Nguyen et al.   MOLECULAR NEUROBIOLOGY

Mutations in Prion Protein Gene: Pathogenic Mechanisms in C-Terminal vs. N-Terminal Domain, a Review

(2019) Livia Bernardi et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Liquid–Liquid Phase Separation Is Driven by Large-Scale Conformational Unwinding and Fluctuations of Intrinsically Disordered Protein Molecules

(2019) Anupa Majumdar et al.   Journal of Physical Chemistry Letters

Liquid‐liquid phase separation and fibrillation of the prion protein modulated by a high‐affinity DNA aptamer

(2019) Carolina O. Matos et al.   FASEB JOURNAL

Energy migration captures membrane-induced oligomerization of the prion protein

(2019) Aishwarya Agarwal et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Demyelinating polyneuropathy in goats lacking prion protein

(2019) Fredrik S. Skedsmo et al.   FASEB JOURNAL

Neutralizing Mutations Significantly Inhibit Amyloid Formation by Human Prion Protein and Decrease Its Cytotoxicity

(2019) Jun-Jie Huang et al.   JOURNAL OF MOLECULAR BIOLOGY

Structural evidence for the critical role of the prion protein hydrophobic region in forming an infectious prion

(2019) Romany Abskharon et al.   PLoS Pathogens

Relationship of Sequence and Phase Separation in Protein Low-Complexity Regions

(2018) Erik W. Martin et al.   BIOCHEMISTRY

Prions, prionoids and protein misfolding disorders

(2018) Claudia Scheckel et al.   NATURE REVIEWS GENETICS

IUPred2A: context-dependent prediction of protein disorder as a function of redox state and protein binding

(2018) Bálint Mészáros et al.   NUCLEIC ACIDS RESEARCH

Protein Phase Separation: A New Phase in Cell Biology

(2018) Steven Boeynaems et al.   TRENDS IN CELL BIOLOGY

Sequence determinants of protein phase behavior from a coarse-grained model

(2018) Gregory L. Dignon et al.   PLoS Computational Biology

Structural basis for the complete resistance of the human prion protein mutant G127V to prion disease

(2018) Zhen Zheng et al.   Scientific Reports

Liquid and Hydrogel Phases of PrPC Linked to Conformation Shifts and Triggered by Alzheimer’s Amyloid-β Oligomers

(2018) Mikhail A. Kostylev et al.   MOLECULAR CELL

Unraveling Prion Protein Interactions with Aptamers and Other PrP-Binding Nucleic Acids

(2017) et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

α-synuclein interacts with PrPC to induce cognitive impairment through mGluR5 and NMDAR2B

(2017) Diana G Ferreira et al.   NATURE NEUROSCIENCE

The N-terminus of the prion protein is a toxic effector regulated by the C-terminus

(2017) Bei Wu et al.   eLife

α-Synuclein Amyloids Hijack Prion Protein to Gain Cell Entry, Facilitate Cell-to-Cell Spreading and Block Prion Replication

(2017) Suzana Aulić et al.   Scientific Reports

The “Jekyll and Hyde” Actions of Nucleic Acids on the Prion-like Aggregation of Proteins

(2016) Jerson L. Silva et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Sequence Determinants of Intracellular Phase Separation by Complex Coacervation of a Disordered Protein

(2016) Chi W. Pak et al.   MOLECULAR CELL

Phase Separation: Linking Cellular Compartmentalization to Disease

(2016) Adriano Aguzzi et al.   TRENDS IN CELL BIOLOGY

Yeast and Fungal Prions

(2016) Reed B. Wickner   Cold Spring Harbor Perspectives in Biology

A Concentration-Dependent Liquid Phase Separation Can Cause Toxicity upon Increased Protein Expression

(2016) Benedetta Bolognesi et al.   Cell Reports

Molecular Mechanism of the Misfolding and Oligomerization of the Prion Protein: Current Understanding and Its Implications

(2015) Jogender Singh et al.   BIOCHEMISTRY

Amyloid-β Receptors: The Good, the Bad, and the Prion Protein

(2015) Heledd H. Jarosz-Griffiths et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2015) Emmanuel A. Asante et al.   NATURE

Polymer physics of intracellular phase transitions

(2015) Clifford P. Brangwynne et al.   Nature Physics

Contrasting Effects of Two Lipid Cofactors of Prion Replication on the Conformation of the Prion Protein

(2015) Saurabh Srivastava et al.   PLoS One

PLAAC: a web and command-line application to identify proteins with prion-like amino acid composition

(2014) A. K. Lancaster et al.   BIOINFORMATICS

Role of lipid in forming an infectious prion?

(2013) F. Wang et al.   ACTA BIOCHIMICA ET BIOPHYSICA SINICA

Biology and Genetics of Prions Causing Neurodegeneration

(2013) Stanley B. Prusiner   Annual Review of Genetics

Conserved amyloid core structure of stop mutants of the human prion protein

(2013) Markus Zweckstetter   Prion

Amyloid-β induced signaling by cellular prion protein and Fyn kinase in Alzheimer disease

(2013) Ji Won Um et al.   Prion

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

(2012) Ivana Biljan et al.   BIOCHEMICAL JOURNAL

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

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

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

Biological and Biochemical Characterization of Mice Expressing Prion Protein Devoid of the Octapeptide Repeat Region after Infection with Prions

(2012) Yoshitaka Yamaguchi et al.   PLoS One

α-Cleavage of cellular prion protein

(2012) Jingjing Liang et al.   Prion

Isolation of phosphatidylethanolamine as a solitary cofactor for prion formation in the absence of nucleic acids

(2012) N. R. Deleault 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 Role of Cofactors in Prion Propagation and Infectivity

(2012) Jiyan Ma   PLoS Pathogens

Prion protein function and the disturbance of early embryonic development in zebrafish

(2011) Mohasina Syed et al.   Prion

Recombinant prion protein induces a new transmissible prion disease in wild-type animals

(2010) Natallia Makarava et al.   ACTA NEUROPATHOLOGICA

PONDR-FIT: A meta-predictor of intrinsically disordered amino acids

(2010) Bin Xue et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

The role of lipid rafts in prion protein biology

(2010) Victoria Lewis   Frontiers in Bioscience-Landmark

Mammalian Prions Generated from Bacterially Expressed Prion Protein in the Absence of Any Mammalian Cofactors

(2010) Jae-Il Kim et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Protease-Sensitive Synthetic Prions

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

Cellular prion protein mediates impairment of synaptic plasticity by amyloid-β oligomers

(2009) Juha Laurén et al.   NATURE

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

An Analytical Solution to the Kinetics of Breakable Filament Assembly

(2009) T. P. J. Knowles et al.   SCIENCE

Molecular Mechanisms of Prion Pathogenesis

(2008) Adriano Aguzzi et al.   Annual Review of Pathology-Mechanisms of Disease

A large ribonucleoprotein particle induced by cytoplasmic PrP shares striking similarities with the chromatoid body, an RNA granule predicted to function in posttranscriptional gene regulation

(2008) Simon Beaudoin et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Molecular conformation and dynamics of the Y145Stop variant of human prion protein in amyloid fibrils

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

Intriguing nucleic-acid-binding features of mammalian prion protein

(2008) Jerson L. Silva et al.   TRENDS IN BIOCHEMICAL SCIENCES