Combating deleterious phase transitions in neurodegenerative disease

Reverse engineering Lewy bodies: how far have we come and how far can we go?

(2021) Mohamed Bilal Fares et al.   NATURE REVIEWS NEUROSCIENCE

Computational insights into mechanism of AIM4-mediated inhibition of aggregation of TDP-43 protein implicated in ALS and evidence for in vitro inhibition of liquid-liquid phase separation (LLPS) of TDP-432C-A315T by AIM4

(2020) Amandeep Girdhar et al.   INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES

The clinical trial landscape in amyotrophic lateral sclerosis—Past, present, and future

(2020) Heike J. Wobst et al.   MEDICINAL RESEARCH REVIEWS

The process of Lewy body formation, rather than simply α-synuclein fibrillization, is one of the major drivers of neurodegeneration

(2020) Anne-Laure Mahul-Mellier et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 α-helical structure tunes liquid–liquid phase separation and function

(2020) Alexander E. Conicella et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structures of α-synuclein filaments from multiple system atrophy

(2020) Manuel Schweighauser et al.   NATURE

Antisense oligonucleotides for neurodegeneration

(2020) Blair R. Leavitt et al.   SCIENCE

Karyopherins and condensates

(2020) Charis E. Springhower et al.   CURRENT OPINION IN CELL BIOLOGY

α-Synuclein aggregation nucleates through liquid–liquid phase separation

(2020) Soumik Ray et al.   Nature Chemistry

Do Lewy bodies contain alpha-synuclein fibrils? and Does it matter? A brief history and critical analysis of recent reports

(2020) Hilal A. Lashuel   NEUROBIOLOGY OF DISEASE

Phase 1–2 Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS

(2020) Timothy Miller et al.   NEW ENGLAND JOURNAL OF MEDICINE

Prion protein lowering is a disease-modifying therapy across prion disease stages, strains and endpoints

(2020) Eric Vallabh Minikel et al.   NUCLEIC ACIDS RESEARCH

Liquid-liquid phase separation induces pathogenic tau conformations in vitro

(2020) Nicholas M. Kanaan et al.   Nature Communications

Tau Ser208 phosphorylation promotes aggregation and reveals neuropathologic diversity in Alzheimer’s disease and other tauopathies

(2020) Yuxing Xia et al.   Acta Neuropathologica Communications

Enzymatic degradation of RNA causes widespread protein aggregation in cell and tissue lysates

(2020) Johan Aarum et al.   EMBO REPORTS

G‐Quadruplexes act as sequence‐dependent protein chaperones

(2020) Adam Begeman et al.   EMBO REPORTS

The prion-like domain of FUS is phosphorylated by multiple kinases affecting liquid- and solid-phase transitions

(2020) Izzy Owen et al.   MOLECULAR BIOLOGY OF THE CELL

Weak binding to the A2RE RNA rigidifies hnRNPA2 RRMs and reduces liquid–liquid phase separation and aggregation

(2020) Veronica H Ryan et al.   NUCLEIC ACIDS RESEARCH

Beyond aggregation: Pathological phase transitions in neurodegenerative disease

(2020) Cécile Mathieu et al.   SCIENCE

Anti-α-synuclein ASO delivered to monoamine neurons prevents α-synuclein accumulation in a Parkinson's disease-like mouse model and in monkeys

(2020) Diana Alarcón-Arís et al.   EBioMedicine

Emerging Roles of Tripartite Motif-Containing Family Proteins (TRIMs) in Eliminating Misfolded Proteins

(2020) Litian Zhang et al.   Frontiers in Cell and Developmental Biology

Ubiquitin signaling in neurodegenerative diseases: an autophagy and proteasome perspective

(2020) François Le Guerroué et al.   CELL DEATH AND DIFFERENTIATION

Acute targeting of pre-amyloid seeds in transgenic mice reduces Alzheimer-like pathology later in life

(2020) Ruth E. Uhlmann et al.   NATURE NEUROSCIENCE

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

Cellular proteostasis decline in human senescence

(2020) Niv Sabath et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Protein condensates as aging Maxwell fluids

(2020) Louise Jawerth et al.   SCIENCE

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

(2020) Guixin Zhu et al.   Cell Reports

Nuclear Import Receptors Directly Bind to Arginine-Rich Dipeptide Repeat Proteins and Suppress Their Pathological Interactions

(2020) Saskia Hutten et al.   Cell Reports

Therapeutic genetic variation revealed in diverse Hsp104 homologs

(2020) Zachary M March et al.   eLife

Treating Disease at the RNA Level with Oligonucleotides

(2019) Arthur A. Levin   NEW ENGLAND JOURNAL OF MEDICINE

Therapeutic Dissolution of Aberrant Phases by Nuclear-Import Receptors

(2019) Lin Guo et al.   TRENDS IN CELL BIOLOGY

Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis

(2019) Archana Prasad et al.   Frontiers in Molecular Neuroscience

Bridging biophysics and neurology: aberrant phase transitions in neurodegenerative disease

(2019) Natalia B. Nedelsky et al.   Nature Reviews Neurology

Matter over mind: Liquid phase separation and neurodegeneration

(2019) Shana Elbaum-Garfinkle   JOURNAL OF BIOLOGICAL CHEMISTRY

Considerations and Challenges in Studying Liquid-Liquid Phase Separation and Biomolecular Condensates

(2019) Simon Alberti et al.   CELL

AAA+ protein-based technologies to counter neurodegenerative disease

(2019) Zachary M. March et al.   BIOPHYSICAL JOURNAL

DNAJC proteins and pathways to parkinsonism

(2019) Dorien A. Roosen et al.   FEBS Journal

Defining α-synuclein species responsible for Parkinson disease phenotypes in mice

(2019) Jessica M. Froula et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

ULK1 and ULK2 Regulate Stress Granule Disassembly Through Phosphorylation and Activation of VCP/p97

(2019) Bo Wang et al.   MOLECULAR CELL

Spontaneous driving forces give rise to protein−RNA condensates with coexisting phases and complex material properties

(2019) Steven Boeynaems et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Nucleocytoplasmic transport defects in neurodegeneration — Cause or consequence?

(2019) Saskia Hutten et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Lou Gehrig’s Disease (ALS): UBQLN2 Mutations Strike Out of Phase

(2019) Nicole Higgins et al.   STRUCTURE

RNA is a critical element for the sizing and the composition of phase-separated RNA–protein condensates

(2019) Marina Garcia-Jove Navarro et al.   Nature Communications

Proteostasis and ALS: protocol for a phase II, randomised, double-blind, placebo-controlled, multicentre clinical trial for colchicine in ALS (Co-ALS)

(2019) Jessica Mandrioli et al.   BMJ Open

Enhancement of the Anti-Aggregation Activity of a Molecular Chaperone Using a Rationally Designed Post-Translational Modification

(2019) Philip R. Lindstedt et al.   ACS Central Science

Parkinson’s disease is a type of amyloidosis featuring accumulation of amyloid fibrils of α-synuclein

(2019) Katsuya Araki et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

“Trim”ming PolyQ proteins with engineered PML

(2019) Neha Dhar et al.   BIOTECHNOLOGY AND BIOENGINEERING

Loss of Dynamic RNA Interaction and Aberrant Phase Separation Induced by Two Distinct Types of ALS/FTD-Linked FUS Mutations

(2019) Amirhossein Ghanbari Niaki et al.   MOLECULAR CELL

Custom therapies pose huge financial burdens

(2019) Carrie Arnold   NATURE MEDICINE

Hsp110 mitigates α-synuclein pathology in vivo

(2019) Yumiko V. Taguchi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Phase separation-deficient TDP43 remains functional in splicing

(2019) Hermann Broder Schmidt et al.   Nature Communications

Low-complexity domain of U1-70K modulates phase separation and aggregation through distinctive basic-acidic motifs

(2019) Song Xue et al.   Science Advances

Emerging small-molecule therapeutic approaches for amyotrophic lateral sclerosis and frontotemporal dementia

(2019) Dean G. Brown et al.   BIOORGANIC & MEDICINAL CHEMISTRY LETTERS

Integrated Stress Response Inhibitor Reverses Sex-Dependent Behavioral and Cell-Specific Deficits after Mild Repetitive Head Trauma

(2019) Karen Krukowski et al.   JOURNAL OF NEUROTRAUMA

Differential α-synuclein expression contributes to selective vulnerability of hippocampal neuron subpopulations to fibril-induced toxicity

(2018) Esteban Luna et al.   ACTA NEUROPATHOLOGICA

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

(2018) Mario Hofweber et al.   CELL

Tau Proteins and Tauopathies in Alzheimer’s Disease

(2018) Fong Ping Chong et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Emerging antisense oligonucleotide and viral therapies for amyotrophic lateral sclerosis

(2018) Cindy V. Ly et al.   CURRENT OPINION IN NEUROLOGY

RNA-binding proteins with basic-acidic dipeptide (BAD) domains self-assemble and aggregate in Alzheimer's disease

(2018) Isaac Bishof et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Randomized, double-blind, placebo-controlled trial of arimoclomol in rapidly progressiveSOD1ALS

(2018) Michael Benatar et al.   NEUROLOGY

Protein Phase Separation: A New Phase in Cell Biology

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

Intrinsic Disorder and Posttranslational Modifications: The Darker Side of the Biological Dark Matter

(2018) April L. Darling et al.   Frontiers in Genetics

The molecular language of membraneless organelles

(2018) Edward Gomes et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Gene therapy for neurological disorders: progress and prospects

(2018) Benjamin E. Deverman et al.   NATURE REVIEWS DRUG DISCOVERY

Friend or foe — post-translational modifications as regulators of phase separation and RNP granule dynamics

(2018) Mario Hofweber 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

Pathways of cellular proteostasis in aging and disease

(2017) Courtney L. Klaips et al.   JOURNAL OF CELL BIOLOGY

Global Post-Translational Modification Discovery

(2017) Qiyao Li et al.   JOURNAL OF PROTEOME RESEARCH

Amyotrophic lateral sclerosis

(2017) Michael A van Es et al.   LANCET

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

(2017) Steven Boeynaems et al.   MOLECULAR CELL

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

(2017) Lindsay A. Becker et al.   NATURE

Reducing the RNA binding protein TIA1 protects against tau-mediated neurodegeneration in vivo

(2017) Daniel J. Apicco et al.   NATURE NEUROSCIENCE

Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31

(2017) Taro Ishiguro et al.   NEURON

Single-Dose Gene-Replacement Therapy for Spinal Muscular Atrophy

(2017) Jerry R. Mendell et al.   NEW ENGLAND JOURNAL OF MEDICINE

Amyotrophic Lateral Sclerosis

(2017) Robert H. Brown et al.   NEW ENGLAND JOURNAL OF MEDICINE

Inhibition of the integrated stress response reverses cognitive deficits after traumatic brain injury

(2017) Austin Chou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Identification of the Tau phosphorylation pattern that drives its aggregation

(2017) Clément Despres et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Special focus on the Cajal Body

(2017) Karla M. Neugebauer   RNA Biology

ATP as a biological hydrotrope

(2017) Avinash Patel et al.   SCIENCE

Liquid–liquid phase separation of the microtubule-binding repeats of the Alzheimer-related protein Tau

(2017) Susmitha Ambadipudi et al.   Nature Communications

Local Nucleation of Microtubule Bundles through Tubulin Concentration into a Condensed Tau Phase

(2017) Amayra Hernández-Vega et al.   Cell Reports

RNA stores tau reversibly in complex coacervates

(2017) Xuemei Zhang et al.   PLOS BIOLOGY

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

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

ATPase-Modulated Stress Granules Contain a Diverse Proteome and Substructure

(2016) Saumya Jain et al.   CELL

Coexisting Liquid Phases Underlie Nucleolar Subcompartments

(2016) Marina Feric et al.   CELL

Genetics of Frontotemporal Dementia

(2016) Diana A. Olszewska et al.   Current Neurology and Neuroscience Reports

The amyloid hypothesis of Alzheimer's disease at 25 years

(2016) Dennis J Selkoe et al.   EMBO Molecular Medicine

The chaperone HSPB8 reduces the accumulation of truncated TDP-43 species in cells and protects against TDP-43-mediated toxicity

(2016) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

Modulation of Intrinsically Disordered Protein Function by Post-translational Modifications

(2016) Alaji Bah 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

Engineering therapeutic protein disaggregases

(2016) James Shorter   MOLECULAR BIOLOGY OF THE CELL

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

(2016) Massimo Ganassi et al.   MOLECULAR CELL

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

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

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110

(2016) Maria Nagy et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

RNAs as chaperones

(2016) Scott Horowitz et al.   RNA Biology

ALS Mutations Disrupt Phase Separation Mediated by α-Helical Structure in the TDP-43 Low-Complexity C-Terminal Domain

(2016) Alexander E. Conicella et al.   STRUCTURE

In Vivo Formation of Vacuolated Multi-phase Compartments Lacking Membranes

(2016) Hermann Broder Schmidt et al.   Cell Reports

Interaction of tau with the RNA-Binding Protein TIA1 Regulates tau Pathophysiology and Toxicity

(2016) Tara Vanderweyde et al.   Cell Reports

Gene delivery of neurturin to putamen and substantia nigra in Parkinson disease: A double-blind, randomized, controlled trial

(2015) C. Warren Olanow et al.   ANNALS OF NEUROLOGY

Nuclear localization signals for four distinct karyopherin-β nuclear import systems

(2015) Michael Soniat et al.   BIOCHEMICAL JOURNAL

A Rational Design Strategy for the Selective Activity Enhancement of a Molecular Chaperone toward a Target Substrate

(2015) Francesco A. Aprile et al.   BIOCHEMISTRY

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

Chaperones in Neurodegeneration

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

Human Hsp70 Disaggregase Reverses Parkinson’s-Linked α-Synuclein Amyloid Fibrils

(2015) Xuechao Gao et al.   MOLECULAR CELL

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

(2015) Yuan Lin 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

Gene therapy returns to centre stage

(2015) Luigi Naldini   NATURE

Crucial HSP70 co-chaperone complex unlocks metazoan protein disaggregation

(2015) Nadinath B. Nillegoda et al.   NATURE

Multisystem proteinopathy: Table

(2015) J. Paul Taylor   NEUROLOGY

Engineering enhanced protein disaggregases for neurodegenerative disease

(2015) Meredith E Jackrel et al.   Prion

The disordered P granule protein LAF-1 drives phase separation into droplets with tunable viscosity and dynamics

(2015) Shana Elbaum-Garfinkle et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Molecular chaperones and neuronal proteostasis

(2015) Heather L. Smith et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

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

(2015) Sonja Kroschwald et al.   eLife

Liquid-Liquid Phase Separation in Biology

(2014) Anthony A. Hyman et al.   Annual Review of Cell and Developmental Biology

Potentiated Hsp104 Variants Antagonize Diverse Proteotoxic Misfolding Events

(2014) Meredith E. Jackrel et al.   CELL

Mechanisms of protein-folding diseases at a glance

(2014) J. S. Valastyan et al.   Disease Models & Mechanisms

Aggregation Properties of the Small Nuclear Ribonucleoprotein U1-70K in Alzheimer Disease

(2014) Ian Diner et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Cellular System that Degrades Misfolded Proteins and Protects against Neurodegeneration

(2014) Lili Guo et al.   MOLECULAR CELL

Allele-Specific Knockdown of ALS-Associated Mutant TDP-43 in Neural Stem Cells Derived from Induced Pluripotent Stem Cells

(2014) Agnes L. Nishimura et al.   PLoS One

The metazoan protein disaggregase and amyloid depolymerase system

(2014) Mariana P Torrente et al.   Prion

Protein disorder, prion propensities, and self-organizing macromolecular collectives

(2013) Liliana Malinovska et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

Stress granules as crucibles of ALS pathogenesis

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

RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention

(2013) Christopher J. Donnelly et al.   NEURON

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

(2013) Shuo-Chien Ling et al.   NEURON

RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia

(2013) T. Zu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration

(2013) C. Lagier-Tourenne et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS

(2013) K. Mori et al.   SCIENCE

RNA Seeds Higher-Order Assembly of FUS Protein

(2013) Jacob C. Schwartz et al.   Cell Reports

The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis

(2013) Yoshinori Nishimoto et al.   Molecular Brain

Hsp104 Suppresses Polyglutamine-Induced Degeneration Post Onset in a Drosophila MJD/SCA3 Model

(2013) Mimi Cushman-Nick et al.   PLoS Genetics

Pharmacological brake-release of mRNA translation enhances cognitive memory

(2013) Carmela Sidrauski et al.   eLife

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

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

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

(2012) Masato Kato et al.   CELL

Contrasting Pathology of the Stress Granule Proteins TIA-1 and G3BP in Tauopathies

(2012) T. Vanderweyde et al.   JOURNAL OF NEUROSCIENCE

α-Synuclein and Neuronal Cell Death

(2012) Toru Yasuda et al.   MOLECULAR NEUROBIOLOGY

Phase transitions in the assembly of multivalent signalling proteins

(2012) Pilong Li et al.   NATURE

The many faces of α-synuclein: from structure and toxicity to therapeutic target

(2012) Hilal A. Lashuel et al.   NATURE REVIEWS NEUROSCIENCE

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

(2012) K. C. Luk et al.   SCIENCE

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

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

The elusive middle domain of Hsp104 and ClpB: Location and function

(2011) Morgan E. DeSantis et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients

(2011) T. Lee et al.   HUMAN MOLECULAR GENETICS

Shock and awe: unleashing the heat shock response to treat Huntington disease

(2011) Meredith E. Jackrel et al.   JOURNAL OF CLINICAL INVESTIGATION

Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease

(2011) John Labbadia et al.   JOURNAL OF CLINICAL INVESTIGATION

AAV2-GAD gene therapy for advanced Parkinson's disease: a double-blind, sham-surgery controlled, randomised trial

(2011) Peter A LeWitt et al.   LANCET NEUROLOGY

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Evaluating the prevalence of polyglutamine repeat expansions in amyotrophic lateral sclerosis

(2011) T. Lee et al.   NEUROLOGY

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

(2011) Laura A. Volpicelli-Daley 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

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

(2011) Zhihui Sun et al.   PLOS BIOLOGY

A role of small heat shock protein B8 (HspB8) in the autophagic removal of misfolded proteins responsible for neurodegenerative diseases

(2010) Valeria Crippa et al.   Autophagy

Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration

(2010) Agnes L. Nishimura et al.   BRAIN

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)

(2010) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

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

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

Directed Evolution of the DnaK Chaperone: Mutations in the Lid Domain Result in Enhanced Chaperone Activity

(2010) Raphael A. Aponte et al.   JOURNAL OF MOLECULAR BIOLOGY

Regulation of TDP-43 aggregation by phosphorylation andp62/SQSTM1

(2010) Owen A. Brady et al.   JOURNAL OF NEUROCHEMISTRY

Emergence and natural selection of drug-resistant prions

(2010) James Shorter   Molecular BioSystems

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

(2010) Andrew C. Elden et al.   NATURE

Tau protein and tau aggregation inhibitors

(2010) Bruno Bulic et al.   NEUROPHARMACOLOGY

Countering amyloid polymorphism and drug resistance with minimal drug cocktails

(2010) Martin L. Duennwald et al.   Prion

Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies

(2009) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

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

Alzheimer's disease

(2009) Lennart Mucke   NATURE

A synergistic small-molecule combination directly eradicates diverse prion strain structures

(2009) Blake E Roberts et al.   Nature Chemical Biology

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

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

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Protein misfolding inside cells: The case of huntingtin and Huntington's disease

(2008) Danny M. Hatters   IUBMB LIFE

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

Vesicle associated membrane protein B (VAPB) is decreased in ALS spinal cord

(2008) Georgia Anagnostou et al.   NEUROBIOLOGY OF AGING

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE