Prion-Like Proteins in Phase Separation and Their Link to Disease

Drug startups coalesce around condensates

(2021) Elie Dolgin   NATURE BIOTECHNOLOGY

Ligand effects on phase separation of multivalent macromolecules

(2021) Kiersten M. Ruff et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Drivers of Hsp104 potentiation revealed by scanning mutagenesis of the middle domain

(2021) Jeremy J. Ryan et al.   PROTEIN SCIENCE

Co-Existence of tau and α-synuclein pathology in fetal graft tissue at autopsy: A case report

(2020) Abbie S. Ornelas et al.   PARKINSONISM & RELATED DISORDERS

Translation of the intrinsically disordered protein α-synuclein is inhibited by a small molecule targeting its structured mRNA

(2020) Peiyuan Zhang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

G3BP1 Is a Tunable Switch that Triggers Phase Separation to Assemble Stress Granules

(2020) Peiguo Yang et al.   CELL

RNA-Induced Conformational Switching and Clustering of G3BP Drive Stress Granule Assembly by Condensation

(2020) Jordina Guillén-Boixet et al.   CELL

α-Synuclein aggregation nucleates through liquid–liquid phase separation

(2020) Soumik Ray et al.   Nature Chemistry

Partitioning of cancer therapeutics in nuclear condensates

(2020) Isaac A. Klein et al.   SCIENCE

C9orf72 poly(GR) aggregation induces TDP-43 proteinopathy

(2020) Casey N. Cook et al.   Science Translational Medicine

TRIM11 Prevents and Reverses Protein Aggregation and Rescues a Mouse Model of Parkinson’s Disease

(2020) Guixin Zhu et al.   Cell Reports

Therapeutic genetic variation revealed in diverse Hsp104 homologs

(2020) Zachary M March et al.   eLife

RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43

(2019) Jacob R. Mann et al.   NEURON

The proteostasis network and its decline in ageing

(2019) Mark S. Hipp et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Engineered protein disaggregases mitigate toxicity of aberrant prion-like fusion proteins underlying sarcoma

(2019) Jeremy J. Ryan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The nucleolus functions as a phase-separated protein quality control compartment

(2019) F. Frottin et al.   SCIENCE

Slow Progressive Accumulation of Oligodendroglial Alpha-Synuclein (α-Syn) Pathology in Synthetic α-Syn Fibril-Induced Mouse Models of Synucleinopathy

(2019) Norihito Uemura et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Mining Disaggregase Sequence Space to Safely Counter TDP-43, FUS, and α-Synuclein Proteotoxicity

(2019) Amber Tariq et al.   Cell Reports

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

Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis

(2019) Luke McAlary et al.   Frontiers in Molecular Neuroscience

Invited Review: The role of prion‐like mechanisms in neurodegenerative diseases

(2019) Z. Jaunmuktane et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Nuclear Import Receptor Inhibits Phase Separation of FUS through Binding to Multiple Sites

(2018) Takuya Yoshizawa et al.   CELL

Phase Separation of FUS Is Suppressed by Its Nuclear Import Receptor and Arginine Methylation

(2018) Mario Hofweber et al.   CELL

Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains

(2018) Lin Guo et al.   CELL

Tau protein liquid–liquid phase separation can initiate tau aggregation

(2018) Susanne Wegmann et al.   EMBO JOURNAL

Prion-like low-complexity sequences: Key regulators of protein solubility and phase behavior

(2018) Titus Franzmann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Profilin reduces aggregation and phase separation of huntingtin N-terminal fragments by preferentially binding to soluble monomers and oligomers

(2018) Ammon E. Posey et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Yeast Prions Compared to Functional Prions and Amyloids

(2018) Reed B. Wickner et al.   JOURNAL OF MOLECULAR BIOLOGY

Structural Ensemble Modulation upon Small-Molecule Binding to Disordered Proteins

(2018) Gabriella T. Heller et al.   JOURNAL OF MOLECULAR BIOLOGY

RNA buffers the phase separation behavior of prion-like RNA binding proteins

(2018) Shovamayee Maharana et al.   SCIENCE

Phase separation of a yeast prion protein promotes cellular fitness

(2018) Titus M. Franzmann et al.   SCIENCE

Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization

(2018) Leeanne McGurk et al.   MOLECULAR CELL

Synthetic Prion Selection and Adaptation

(2018) Edoardo Bistaffa et al.   MOLECULAR NEUROBIOLOGY

Nuclear poly(ADP-ribose) activity is a therapeutic target in amyotrophic lateral sclerosis

(2018) L. McGurk et al.   Acta Neuropathologica Communications

TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates

(2018) Florent Laferrière et al.   NATURE NEUROSCIENCE

Structure of Calcarisporiella thermophila Hsp104 Disaggregase that Antagonizes Diverse Proteotoxic Misfolding Events

(2018) Karolina Michalska et al.   STRUCTURE

Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo

(2018) Sílvia Porta et al.   Nature Communications

FUS inclusions disrupt RNA localization by sequestering kinesin-1 and inhibiting microtubule detyrosination

(2017) Kyota Yasuda et al.   JOURNAL OF CELL BIOLOGY

Phase behaviour of disordered proteins underlying low density and high permeability of liquid organelles

(2017) Ming-Tzo Wei et al.   Nature Chemistry

Targeting microbial biofilms: current and prospective therapeutic strategies

(2017) Hyun Koo et al.   NATURE REVIEWS MICROBIOLOGY

Biomolecular condensates: organizers of cellular biochemistry

(2017) Salman F. Banani et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Lost in Transportation: Nucleocytoplasmic Transport Defects in ALS and Other Neurodegenerative Diseases

(2017) Hong Joo Kim et al.   NEURON

Supersaturated proteins in ALS

(2017) Elliott Hayden et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Spinal motor neuron protein supersaturation patterns are associated with inclusion body formation in ALS

(2017) Prajwal Ciryam et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ATP as a biological hydrotrope

(2017) Avinash Patel et al.   SCIENCE

Ratchet-like polypeptide translocation mechanism of the AAA+ disaggregase Hsp104

(2017) Stephanie N. Gates et al.   SCIENCE

Liquid phase condensation in cell physiology and disease

(2017) Yongdae Shin et al.   SCIENCE

Clinical Neurology and Epidemiology of the Major Neurodegenerative Diseases

(2017) Michael G. Erkkinen et al.   Cold Spring Harbor Perspectives in Biology

Semen amyloids participate in spermatozoa selection and clearance

(2017) Nadia R Roan et al.   eLife

Protein-Remodeling Factors As Potential Therapeutics for Neurodegenerative Disease

(2017) Meredith E. Jackrel et al.   Frontiers in Neuroscience

Prion-like domains as epigenetic regulators, scaffolds for subcellular organization, and drivers of neurodegenerative disease

(2016) Zachary M. March et al.   BRAIN RESEARCH

Amyloid-like Self-Assembly of a Cellular Compartment

(2016) Elvan Boke et al.   CELL

Polar Positioning of Phase-Separated Liquid Compartments in Cells Regulated by an mRNA Competition Mechanism

(2016) Shambaditya Saha et al.   CELL

ATPase-Modulated Stress Granules Contain a Diverse Proteome and Substructure

(2016) Saumya Jain et al.   CELL

C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles

(2016) Kyung-Ha Lee et al.   CELL

Toxic PR Poly-Dipeptides Encoded by the C9orf72 Repeat Expansion Target LC Domain Polymers

(2016) Yi Lin et al.   CELL

The balbiani body and the concept of physiological amyloids

(2016) Elvan Boke et al.   CELL CYCLE

Amyloid Structures as Biofilm Matrix Scaffolds

(2016) Agustina Taglialegna et al.   JOURNAL OF BACTERIOLOGY

Mechanistic Insights into Hsp104 Potentiation

(2016) Mariana P. Torrente et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Sam68 nuclear body is composed of two RNase-sensitive substructures joined by the adaptor HNRNPL

(2016) Taro Mannen et al.   JOURNAL OF CELL BIOLOGY

Widespread transneuronal propagation of α-synucleinopathy triggered in olfactory bulb mimics prodromal Parkinson’s disease

(2016) Nolwen L. Rey et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Amyloid formation: functional friend or fearful foe?

(2016) P. Bergman et al.   JOURNAL OF INTERNAL MEDICINE

A Surveillance Function of the HSPB8-BAG3-HSP70 Chaperone Complex Ensures Stress Granule Integrity and Dynamism

(2016) Massimo Ganassi et al.   MOLECULAR CELL

Biofilms: an emergent form of bacterial life

(2016) Hans-Curt Flemming et al.   NATURE REVIEWS MICROBIOLOGY

Spiral architecture of the Hsp104 disaggregase reveals the basis for polypeptide translocation

(2016) Adam L Yokom et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Protein-RNA Networks Regulated by Normal and ALS-Associated Mutant HNRNPA2B1 in the Nervous System

(2016) Fernando J. Martinez et al.   NEURON

Prions, Chaperones, and Proteostasis in Yeast

(2016) Tatiana A. Chernova et al.   Cold Spring Harbor Perspectives in Biology

Spiral architecture of the Hsp104 disaggregase reveals the basis for polypeptide translocation

(2016) Adam L Yokom et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

NMNAT2:HSP90 Complex Mediates Proteostasis in Proteinopathies

(2016) Yousuf O. Ali et al.   PLOS BIOLOGY

ATPase activity of the DEAD-box protein Dhh1 controls processing body formation

(2016) Christopher Frederick Mugler et al.   eLife

Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD

(2016) Steven Boeynaems et al.   Scientific Reports

Disparate Mutations Confer Therapeutic Gain of Hsp104 Function

(2015) Meredith E. Jackrel et al.   ACS Chemical Biology

Amyloidogenic Oligomerization Transforms Drosophila Orb2 from a Translation Repressor to an Activator

(2015) Mohammed Repon Khan et al.   CELL

The LC Domain of hnRNPA2 Adopts Similar Conformations in Hydrogel Polymers, Liquid-like Droplets, and Nuclei

(2015) Siheng Xiang et al.   CELL

Phase Separation by Low Complexity Domains Promotes Stress Granule Assembly and Drives Pathological Fibrillization

(2015) Amandine Molliex et al.   CELL

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

(2015) Avinash Patel et al.   CELL

Biofilm formation mechanisms and targets for developing antibiofilm agents

(2015) Nira Rabin et al.   Future Medicinal Chemistry

The Kringle-like Domain Facilitates Post-endoplasmic Reticulum Changes to Premelanosome Protein (PMEL) Oligomerization and Disulfide Bond Configuration and Promotes Amyloid Formation

(2015) Tina Ho et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prion-like domains in RNA binding proteins are essential for building subnuclear paraspeckles

(2015) Sven Hennig et al.   JOURNAL OF CELL BIOLOGY

TDP-43 is intercellularly transmitted across axon terminals

(2015) Marisa S. Feiler et al.   JOURNAL OF CELL BIOLOGY

RNA Controls PolyQ Protein Phase Transitions

(2015) Huaiying Zhang et al.   MOLECULAR CELL

Formation and Maturation of Phase-Separated Liquid Droplets by RNA-Binding Proteins

(2015) Yuan Lin et al.   MOLECULAR CELL

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

Determinants of amyloid fibril degradation by the PDZ protease HTRA1

(2015) Simon Poepsel et al.   Nature Chemical Biology

Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

The Persistence of Hippocampal-Based Memory Requires Protein Synthesis Mediated by the Prion-like Protein CPEB3

(2015) Luana Fioriti et al.   NEURON

ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function

(2015) Tetsuro Murakami et al.   NEURON

Review: Prion-like mechanisms of transactive response DNA binding protein of 43 kDa (TDP-43) in amyotrophic lateral sclerosis (ALS)

(2015) Phillip Smethurst et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Engineering enhanced protein disaggregases for neurodegenerative disease

(2015) Meredith E Jackrel et al.   Prion

Evidence for α-synuclein prions causing multiple system atrophy in humans with parkinsonism

(2015) Stanley B. Prusiner et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

RNA transcription modulates phase transition-driven nuclear body assembly

(2015) Joel Berry et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits

(2015) J. Chew et al.   SCIENCE

Liquid demixing of intrinsically disordered proteins is seeded by poly(ADP-ribose)

(2015) Matthias Altmeyer et al.   Nature Communications

SUMOylation Is an Inhibitory Constraint that Regulates the Prion-like Aggregation and Activity of CPEB3

(2015) Bettina Drisaldi et al.   Cell Reports

The CPEB3 Protein Is a Functional Prion that Interacts with the Actin Cytoskeleton

(2015) Joseph S. Stephan et al.   Cell Reports

Promiscuous interactions and protein disaggregases determine the material state of stress-inducible RNP granules

(2015) Sonja Kroschwald et al.   eLife

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

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

Potentiated Hsp104 Variants Antagonize Diverse Proteotoxic Misfolding Events

(2014) Meredith E. Jackrel et al.   CELL

Reversing deleterious protein aggregation with re-engineered protein disaggregases

(2014) Meredith E Jackrel et al.   CELL CYCLE

Classification of Intrinsically Disordered Regions and Proteins

(2014) Robin van der Lee et al.   CHEMICAL REVIEWS

Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins

(2014) M. E. Jackrel et al.   Disease Models & Mechanisms

Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases

(2014) Jing L Guo et al.   NATURE MEDICINE

Addition of exogenous α-synuclein preformed fibrils to primary neuronal cultures to seed recruitment of endogenous α-synuclein to Lewy body and Lewy neurite–like aggregates

(2014) Laura A Volpicelli-Daley et al.   Nature Protocols

Distinct Tau Prion Strains Propagate in Cells and Mice and Define Different Tauopathies

(2014) David W. Sanders et al.   NEURON

The metazoan protein disaggregase and amyloid depolymerase system

(2014) Mariana P Torrente et al.   Prion

Genome-wide Translational Changes Induced by the Prion [PSI+]

(2014) Agnès Baudin-Baillieu et al.   Cell Reports

Conformational Features of Tau Fibrils from Alzheimer’s Disease Brain Are Faithfully Propagated by Unmodified Recombinant Protein

(2013) Olga A. Morozova et al.   BIOCHEMISTRY

Prion-like spreading of pathological α-synuclein in brain

(2013) Masami Masuda-Suzukake et al.   BRAIN

Eukaryotic Stress Granules Are Cleared by Autophagy and Cdc48/VCP Function

(2013) J. Ross Buchan et al.   CELL

Distinct α-Synuclein Strains Differentially Promote Tau Inclusions in Neurons

(2013) Jing L. Guo et al.   CELL

Seeded strain-like transmission of β-amyloid morphotypes in APP transgenic mice

(2013) Götz Heilbronner et al.   EMBO REPORTS

Stress granules as crucibles of ALS pathogenesis

(2013) Yun R. Li et al.   JOURNAL OF CELL BIOLOGY

Synthetic Tau Fibrils Mediate Transmission of Neurofibrillary Tangles in a Transgenic Mouse Model of Alzheimer's-Like Tauopathy

(2013) M. Iba et al.   JOURNAL OF NEUROSCIENCE

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Chaperone machines for protein folding, unfolding and disaggregation

(2013) Helen Saibil   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Unmasking the roles of N- and C-terminal flanking sequences from exon 1 of huntingtin as modulators of polyglutamine aggregation

(2013) S. L. Crick et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Blessings in disguise: biological benefits of prion-like mechanisms

(2013) Gregory A. Newby et al.   TRENDS IN CELL BIOLOGY

Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains

(2013) Takashi Nonaka et al.   Cell Reports

Hexanucleotide Repeats in ALS/FTD Form Length-Dependent RNA Foci, Sequester RNA Binding Proteins, and Are Neurotoxic

(2013) Youn-Bok Lee et al.   Cell Reports

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

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

Operational Plasticity Enables Hsp104 to Disaggregate Diverse Amyloid and Nonamyloid Clients

(2012) Morgan E. DeSantis et al.   CELL

Cell-free Formation of RNA Granules: Low Complexity Sequence Domains Form Dynamic Fibers within Hydrogels

(2012) Masato Kato et al.   CELL

Getting RNA and Protein in Phase

(2012) Stephanie C. Weber et al.   CELL

The Amyloid State of Proteins in Human Diseases

(2012) David Eisenberg et al.   CELL

Metazoan Hsp70 machines use Hsp110 to power protein disaggregation

(2012) Heike Rampelt et al.   EMBO JOURNAL

Requirements for Stress Granule Recruitment of Fused in Sarcoma (FUS) and TAR DNA-binding Protein of 43 kDa (TDP-43)

(2012) Eva Bentmann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Intracerebral inoculation of pathological α-synuclein initiates a rapidly progressive neurodegenerative α-synucleinopathy in mice

(2012) Kelvin C. Luk et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Prions are a common mechanism for phenotypic inheritance in wild yeasts

(2012) Randal Halfmann et al.   NATURE

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

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

D2P2: database of disordered protein predictions

(2012) Matt E. Oates et al.   NUCLEIC ACIDS RESEARCH

Pathological  -Synuclein Transmission Initiates Parkinson-like Neurodegeneration in Nontransgenic Mice

(2012) K. C. Luk et al.   SCIENCE

P-Bodies and Stress Granules: Possible Roles in the Control of Translation and mRNA Degradation

(2012) C. J. Decker et al.   Cold Spring Harbor Perspectives in Biology

Small Heat Shock Proteins Potentiate Amyloid Dissolution by Protein Disaggregases from Yeast and Humans

(2012) Martin L. Duennwald et al.   PLOS BIOLOGY

Neuralized1 Activates CPEB3: A Function for Nonproteolytic Ubiquitin in Synaptic Plasticity and Memory Storage

(2011) Elias Pavlopoulos et al.   CELL

Understanding the role of TDP-43 and FUS/TLS in ALS and beyond

(2011) Sandrine Da Cruz et al.   CURRENT OPINION IN NEUROBIOLOGY

A Seeding Reaction Recapitulates Intracellular Formation of Sarkosyl-insoluble Transactivation Response Element (TAR) DNA-binding Protein-43 Inclusions

(2011) Yoshiaki Furukawa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Seeding of Normal Tau by Pathological Tau Conformers Drives Pathogenesis of Alzheimer-like Tangles

(2011) Jing L. Guo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

P granules extend the nuclear pore complex environment in theC. elegansgerm line

(2011) Dustin L. Updike et al.   JOURNAL OF CELL BIOLOGY

Exogenous α-Synuclein Fibrils Induce Lewy Body Pathology Leading to Synaptic Dysfunction and Neuron Death

(2011) Laura A. Volpicelli-Daley et al.   NEURON

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

The Mammalian Disaggregase Machinery: Hsp110 Synergizes with Hsp70 and Hsp40 to Catalyze Protein Disaggregation and Reactivation in a Cell-Free System

(2011) James Shorter   PLoS One

Active liquid-like behavior of nucleoli determines their size and shape in Xenopus laevis oocytes

(2011) C. P. Brangwynne et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A yeast functional screen predicts new candidate ALS disease genes

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

Suicidal [PSI+] is a lethal yeast prion

(2011) R. P. McGlinchey et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Neurotoxic effects of TDP-43 overexpression in C. elegans

(2010) Peter E.A. Ash et al.   HUMAN MOLECULAR GENETICS

Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules

(2010) Daryl A. Bosco et al.   HUMAN MOLECULAR GENETICS

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration

(2010) C. Lagier-Tourenne et al.   HUMAN MOLECULAR GENETICS

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden et al.   NATURE

The prion hypothesis: from biological anomaly to basic regulatory mechanism

(2010) Mick F. Tuite et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

TAR DNA-binding protein 43 in neurodegenerative disease

(2010) Alice S. Chen-Plotkin et al.   Nature Reviews Neurology

TDP-43-Mediated Neuron Loss In Vivo Requires RNA-Binding Activity

(2010) Aaron Voigt et al.   PLoS One

Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue

(2010) Liqun Liu-Yesucevitz et al.   PLoS One

Prion proteostasis

(2010) Elizabeth A. Sweeny et al.   Prion

A Systematic Survey Identifies Prions and Illuminates Sequence Features of Prionogenic Proteins

(2009) Simon Alberti et al.   CELL

Prion protein misfolding and disease

(2009) Roger A Moore et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

N-terminal Domains Elicit Formation of Functional Pmel17 Amyloid Fibrils

(2009) Brenda Watt et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity

(2009) Brian S. Johnson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Robust heat shock induces eIF2 -phosphorylation-independent assembly of stress granules containing eIF3 and 40S ribosomal subunits in budding yeast, Saccharomyces cerevisiae

(2009) T. Grousl et al.   JOURNAL OF CELL SCIENCE

TDP-43 localizes in mRNA transcription and processing sites in mammalian neurons

(2009) Iñigo Casafont et al.   JOURNAL OF STRUCTURAL BIOLOGY

Transmission and spreading of tauopathy in transgenic mouse brain

(2009) Florence Clavaguera et al.   NATURE CELL BIOLOGY

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

Germline P Granules Are Liquid Droplets That Localize by Controlled Dissolution/Condensation

(2009) C. P. Brangwynne et al.   SCIENCE

Functional Amyloids As Natural Storage of Peptide Hormones in Pituitary Secretory Granules

(2009) S. K. Maji et al.   SCIENCE

Robustness: mechanisms and consequences

(2009) Joanna Masel et al.   TRENDS IN GENETICS

Small-molecule perturbation of competing interactions between c-Myc and Max

(2008) Ariele Viacava Follis et al.   BIOORGANIC & MEDICINAL CHEMISTRY LETTERS

Hsp104, Hsp70 and Hsp40 interplay regulates formation, growth and elimination of Sup35 prions

(2008) James Shorter et al.   EMBO JOURNAL

Dynamics of component exchange at PML nuclear bodies

(2008) S. Weidtkamp-Peters et al.   JOURNAL OF CELL SCIENCE

Hsp104 antagonizes α-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease

(2008) Christophe Lo Bianco et al.   JOURNAL OF CLINICAL INVESTIGATION

Live Cell Dynamics of Promyelocytic Leukemia Nuclear Bodies upon Entry into and Exit from Mitosis

(2008) Yi-Chun M. Chen et al.   MOLECULAR BIOLOGY OF THE CELL

Epigenetic control of polyamines by the prion [PSI+]

(2008) Olivier Namy et al.   NATURE CELL BIOLOGY

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity

(2008) B. S. Johnson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE