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

Spiraling in Control: Structures and Mechanisms of the Hsp104 Disaggregase

(2019) James Shorter et al.   Cold Spring Harbor Perspectives in Biology

An Intramolecular Salt Bridge Linking TDP43 RNA Binding, Protein Stability, and TDP43-Dependent Neurodegeneration

(2019) Brittany N. Flores et al.   Cell Reports

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

(2018) Lin Guo et al.   CELL

Amyloid assembly and disassembly

(2018) Edward Chuang et al.   JOURNAL OF CELL SCIENCE

Protein-Based Inheritance: Epigenetics beyond the Chromosome

(2018) Zachary H. Harvey et al.   MOLECULAR CELL

Protein Phase Separation: A New Phase in Cell Biology

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

Molecular Dissection of FUS Points at Synergistic Effect of Low-Complexity Domains in Toxicity

(2018) Elke Bogaert et al.   Cell Reports

Different Material States of Pub1 Condensates Define Distinct Modes of Stress Adaptation and Recovery

(2018) Sonja Kroschwald et al.   Cell Reports

Heat shock protein 104 (Hsp104)-mediated curing of [PSI+] yeast prions depends on both [PSI+] conformation and the properties of the Hsp104 homologs

(2017) Xiaohong Zhao et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice

(2017) Lindsay A. Becker et al.   NATURE

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

A dominant-negative mutant inhibits multiple prion variants through a common mechanism

(2017) Fen Pei et al.   PLoS Genetics

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

Mechanistic Insights into Hsp104 Potentiation

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

Mechanistic and Structural Insights into the Prion-Disaggregase Activity of Hsp104

(2016) Elizabeth A. Sweeny et al.   JOURNAL OF MOLECULAR BIOLOGY

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

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

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

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

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

Biology and Pathobiology of TDP-43 and Emergent Therapeutic Strategies

(2016) Lin Guo et al.   Cold Spring Harbor Perspectives in Medicine

Disparate Mutations Confer Therapeutic Gain of Hsp104 Function

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

Reversible, Specific, Active Aggregates of Endogenous Proteins Assemble upon Heat Stress

(2015) Edward W.J. Wallace et al.   CELL

Chaperones in Neurodegeneration

(2015) I. Lindberg et al.   JOURNAL OF NEUROSCIENCE

The Hsp104 N-Terminal Domain Enables Disaggregase Plasticity and Potentiation

(2015) Elizabeth A. Sweeny et al.   MOLECULAR CELL

It’s Raining Liquids: RNA Tunes Viscoelasticity and Dynamics of Membraneless Organelles

(2015) Lin Guo et al.   MOLECULAR CELL

Residue-by-Residue View of In Vitro FUS Granules that Bind the C-Terminal Domain of RNA Polymerase II

(2015) Kathleen A. Burke et al.   MOLECULAR CELL

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

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

Engineering enhanced protein disaggregases for neurodegenerative disease

(2015) Meredith E Jackrel et al.   Prion

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

(2015) Sonja Kroschwald et al.   eLife

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

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

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

Hsp104 Overexpression Cures Saccharomyces cerevisiae [ PSI + ] by Causing Dissolution of the Prion Seeds

(2014) Yang-Nim Park et al.   EUKARYOTIC CELL

Spatial quality control bypasses cell-based limitations on proteostasis to promote prion curing

(2014) Courtney L Klaips et al.   eLife

Isolating Potentiated Hsp104 Variants Using Yeast Proteinopathy Models

(2014) Meredith E. Jackrel et al.   Jove-Journal of Visualized Experiments

Phosphorylation-Regulated Binding of RNA Polymerase II to Fibrous Polymers of Low-Complexity Domains

(2013) Ilmin Kwon et al.   CELL

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

Identification and Rescue of  -Synuclein Toxicity in Parkinson Patient-Derived Neurons

(2013) C. Y. Chung et al.   SCIENCE

Yeast Reveal a "Druggable" Rsp5/Nedd4 Network that Ameliorates  -Synuclein Toxicity in Neurons

(2013) D. F. Tardiff et al.   SCIENCE

Blessings in disguise: biological benefits of prion-like mechanisms

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

Transportin 1 accumulates specifically with FET proteins but no other transportin cargos in FTLD-FUS and is absent in FUS inclusions in ALS with FUS mutations

(2012) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

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

Hsp104 Drives “Protein-Only” Positive Selection of Sup35 Prion Strains Encoding Strong [PSI+]

(2012) Morgan E. DeSantis et al.   CHEMISTRY & BIOLOGY

Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis

(2012) Julien Couthouis et al.   HUMAN MOLECULAR GENETICS

RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations

(2012) J. G. Daigle et al.   HUMAN MOLECULAR GENETICS

FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations

(2011) Manuela Neumann et al.   BRAIN

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

Functional Links Between A  Toxicity, Endocytic Trafficking, and Alzheimer's Disease Risk Factors in Yeast

(2011) S. Treusch et al.   SCIENCE

A Yeast Model of FUS/TLS-Dependent Cytotoxicity

(2011) Shulin Ju et al.   PLOS BIOLOGY

Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS

(2011) Zhihui Sun et al.   PLOS BIOLOGY

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

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

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

(2010) Andrew C. Elden et al.   NATURE

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

(2010) Aaron Voigt et al.   PLoS One

Prions, protein homeostasis, and phenotypic diversity

(2010) Randal Halfmann et al.   TRENDS IN CELL BIOLOGY

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

The TET Family of Proteins: Functions and Roles in Disease

(2009) A. Y. Tan et al.   Journal of Molecular Cell Biology

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

(2008) James Shorter et al.   EMBO JOURNAL

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

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

Nucleocytoplasmic Trafficking of the Molecular Chaperone Hsp104 in Unstressed and Heat-Shocked Cells

(2007) Johnny M. Tkach et al.   TRAFFIC