(Dis)Solving the problem of aberrant protein states

Does protein aggregation drive postmitotic tissue degeneration?

(2021) Jeffery W. Kelly   Science Translational Medicine

FUS and TDP-43 Phases in Health and Disease

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

Mechanism of karyopherin-β2 binding and nuclear import of ALS variants FUS(P525L) and FUS(R495X)

(2021) Abner Gonzalez et al.   Scientific Reports

Nucleolar stress in C9orf72 and sporadic ALS spinal motor neurons precedes TDP-43 mislocalization

(2021) Olubankole Aladesuyi Arogundade et al.   Acta Neuropathologica Communications

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

Glia-to-Neuron Conversion by CRISPR-CasRx Alleviates Symptoms of Neurological Disease in Mice

(2020) Haibo Zhou et al.   CELL

AAA+ proteins

(2020) Thiago V. Seraphim et al.   CURRENT BIOLOGY

The HSP110/HSP70 disaggregation system generates spreading‐competent toxic α‐synuclein species

(2020) Jessica Tittelmeier et al.   EMBO JOURNAL

Recent advances in understanding catalysis of protein folding by molecular chaperones

(2020) David Balchin et al.   FEBS LETTERS

Structural and kinetic basis for the regulation and potentiation of Hsp104 function

(2020) Xiang Ye et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Reversing a model of Parkinson’s disease with in situ converted nigral neurons

(2020) Hao Qian et al.   NATURE

Proteome-wide identification of HSP70/HSC70 chaperone clients in human cells

(2020) Seung W. Ryu et al.   PLOS BIOLOGY

Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations

(2020) Ryan R Cupo et al.   eLife

Antisense Drugs Make Sense for Neurological Diseases

(2020) C. Frank Bennett et al.   Annual Review of Pharmacology and Toxicology

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

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

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

Development of vaccines and antivirals for combating viral pandemics

(2020) Norbert Pardi et al.   Nature Biomedical Engineering

Therapeutic Dissolution of Aberrant Phases by Nuclear-Import Receptors

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

Spiraling in Control: Structures and Mechanisms of the Hsp104 Disaggregase

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

RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43

(2019) Jacob R. Mann et al.   NEURON

Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death

(2019) Fatima Gasset-Rosa et al.   NEURON

Emerging RNA-binding roles in the TRIM family of ubiquitin ligases

(2019) Felix Preston Williams et al.   BIOLOGICAL CHEMISTRY

Targeting mitochondrial structure sensitizes acute myeloid leukemia to Venetoclax treatment

(2019) Xufeng Chen et al.   Cancer Discovery

Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology

(2019) Peipei Zhang et al.   eLife

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

(2019) F. Frottin et al.   SCIENCE

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

(2019) Amber Tariq et al.   Cell Reports

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

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

The mutational landscape of a prion-like domain

(2019) Benedetta Bolognesi et al.   Nature Communications

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

FUS Phase Separation Is Modulated by a Molecular Chaperone and Methylation of Arginine Cation-π Interactions

(2018) Seema Qamar et al.   CELL

Amyloid assembly and disassembly

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

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

(2018) Ching-Chieh Chou et al.   NATURE NEUROSCIENCE

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

(2018) Shovamayee Maharana et al.   SCIENCE

Protein Phase Separation: A New Phase in Cell Biology

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

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

TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle

(2018) Thomas O. Vogler et al.   NATURE

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

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

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

A scoring system predicting the clinical course of CLPB defect based on the foetal and neonatal presentation of 31 patients

(2017) Ewa Pronicka et al.   JOURNAL OF INHERITED METABOLIC DISEASE

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

(2017) Hong Joo Kim et al.   NEURON

The case for defined protein folding pathways

(2017) S. Walter Englander et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Evolution of an intricate J-protein network driving protein disaggregation in eukaryotes

(2017) Nadinath B Nillegoda et al.   eLife

Engineering therapeutic protein disaggregases

(2016) James Shorter   MOLECULAR BIOLOGY OF THE 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

Engineering enhanced protein disaggregases for neurodegenerative disease

(2015) Meredith E Jackrel et al.   Prion

Binding of TDP-43 to the 3′UTR of Its Cognate mRNA Enhances Its Solubility

(2014) Yulong Sun et al.   BIOCHEMISTRY

Potentiated Hsp104 Variants Antagonize Diverse Proteotoxic Misfolding Events

(2014) Meredith E. Jackrel et al.   CELL

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

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

A Cellular System that Degrades Misfolded Proteins and Protects against Neurodegeneration

(2014) Lili Guo et al.   MOLECULAR CELL

The metazoan protein disaggregase and amyloid depolymerase system

(2014) Mariana P Torrente et al.   Prion

Stress granules as crucibles of ALS pathogenesis

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

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

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

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

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

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

Molecular Mechanism of Protein Folding in the Cell

(2011) James E. Rothman et al.   CELL

Expression of therapeutic proteins after delivery of chemically modified mRNA in mice

(2011) Michael S D Kormann et al.   NATURE BIOTECHNOLOGY

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

Protein Homeostasis and the Phenotypic Manifestation of Genetic Diversity: Principles and Mechanisms

(2010) Daniel F. Jarosz et al.   Annual Review of Genetics

TDP-43 regulates its mRNA levels through a negative feedback loop

(2010) Youhna M Ayala et al.   EMBO JOURNAL

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

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

Emergence and natural selection of drug-resistant prions

(2010) James Shorter   Molecular BioSystems

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