Stress granules and neurodegeneration

ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair

(2019) Joseph R. Klim et al.   NATURE NEUROSCIENCE

Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration

(2019) Ze’ev Melamed et al.   NATURE NEUROSCIENCE

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

The Evolution of Phase-Separated TDP-43 in Stress

(2019) Benjamin Wolozin   NEURON

NIA-AA Research Framework: Toward a biological definition of Alzheimer's disease

(2018) Clifford R. Jack et al.   Alzheimers & Dementia

Untangling Genetic Risk for Alzheimer’s Disease

(2018) Anna A. Pimenova et al.   BIOLOGICAL PSYCHIATRY

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

(2018) Mario Hofweber et al.   CELL

Context-Dependent and Disease-Specific Diversity in Protein Interactions within Stress Granules

(2018) Sebastian Markmiller et al.   CELL

A Molecular Grammar Governing the Driving Forces for Phase Separation of Prion-like RNA Binding Proteins

(2018) Jie Wang et al.   CELL

Stress Granule Assembly Disrupts Nucleocytoplasmic Transport

(2018) Ke Zhang 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

Tau protein liquid–liquid phase separation can initiate tau aggregation

(2018) Susanne Wegmann et al.   EMBO JOURNAL

TIA1 variant drives myodegeneration in multisystem proteinopathy with SQSTM1 mutations

(2018) YouJin Lee et al.   JOURNAL OF CLINICAL INVESTIGATION

Novel genes associated with amyotrophic lateral sclerosis: diagnostic and clinical implications

(2018) Ruth Chia et al.   LANCET NEUROLOGY

High-Density Proximity Mapping Reveals the Subcellular Organization of mRNA-Associated Granules and Bodies

(2018) Ji-Young Youn et al.   MOLECULAR CELL

Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis

(2018) Yong-Jie Zhang et al.   NATURE MEDICINE

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

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

Genome-wide Analyses Identify KIF5A as a Novel ALS Gene

(2018) Aude Nicolas et al.   NEURON

RNA self-assembly contributes to stress granule formation and defining the stress granule transcriptome

(2018) Briana Van Treeck et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2018) Shovamayee Maharana et al.   SCIENCE

Atomic structures of low-complexity protein segments reveal kinked β sheets that assemble networks

(2018) Michael P. Hughes et al.   SCIENCE

A liquid phase of synapsin and lipid vesicles

(2018) Dragomir Milovanovic et al.   SCIENCE

Protein Phase Separation: A New Phase in Cell Biology

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

ALS Genes in the Genomic Era and their Implications for FTD

(2018) Hung Phuoc Nguyen et al.   TRENDS IN GENETICS

Role of eIF2α Kinases in Translational Control and Adaptation to Cellular Stress

(2018) Ronald C. Wek   Cold Spring Harbor Perspectives in 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

Tau Protein Disrupts Nucleocytoplasmic Transport in Alzheimer’s Disease

(2018) Bahareh Eftekharzadeh et al.   NEURON

RNA binding proteins co-localize with small tau inclusions in tauopathy

(2018) Brandon F. Maziuk et al.   Acta Neuropathologica Communications

TIA1 regulates the generation and response to toxic tau oligomers

(2018) Lulu Jiang et al.   ACTA NEUROPATHOLOGICA

Dysregulation of autophagy and stress granule-related proteins in stress-driven Tau pathology

(2018) Joana Margarida Silva et al.   CELL DEATH AND DIFFERENTIATION

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

(2018) Thomas O. Vogler et al.   NATURE

Heavy Metal Neurotoxicants Induce ALS-Linked TDP-43 Pathology

(2018) Peter E A Ash et al.   TOXICOLOGICAL SCIENCES

Isolation of yeast and mammalian stress granule cores

(2017) Joshua R. Wheeler et al.   METHODS

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

(2017) Steven Boeynaems et al.   MOLECULAR CELL

TREM2 deficiency exacerbates tau pathology through dysregulated kinase signaling in a mouse model of tauopathy

(2017) Shane M. Bemiller et al.   Molecular Neurodegeneration

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

(2017) Lindsay A. Becker et al.   NATURE

Cryo-EM structures of tau filaments from Alzheimer’s disease

(2017) Anthony W. P. Fitzpatrick et al.   NATURE

RNA phase transitions in repeat expansion disorders

(2017) Ankur Jain et al.   NATURE

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

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

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

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

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

(2017) Ian R. Mackenzie et al.   NEURON

Stress granule-associated protein G3BP2 regulates breast tumor initiation

(2017) Nisha Gupta et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation

(2017) Tariq Afroz et al.   Nature Communications

ALS/FTD-Associated C9ORF72 Repeat RNA Promotes Phase Transitions In Vitro and in Cells

(2017) Marta M. Fay et al.   Cell Reports

Mammalian ataxin-2 modulates translation control at the pre-initiation complex via PI3K/mTOR and is induced by starvation

(2016) Isabel Lastres-Becker et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Distinct recruitment of human eIF4E isoforms to processing bodies and stress granules

(2016) Klara Frydryskova et al.   BMC MOLECULAR BIOLOGY

The unfolded protein response: mechanisms and therapy of neurodegeneration

(2016) Heather L. Smith et al.   BRAIN

Immunoprecipitation and mass spectrometry defines an extensive RBM45 protein–protein interaction network

(2016) Yang Li et al.   BRAIN RESEARCH

Mechanisms and Consequences of Macromolecular Phase Separation

(2016) Louis-Philippe Bergeron-Sandoval 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

Compositional Control of Phase-Separated Cellular Bodies

(2016) Salman F. Banani et al.   CELL

Coexisting Liquid Phases Underlie Nucleolar Subcompartments

(2016) Marina Feric et al.   CELL

G3BP–Caprin1–USP10 complexes mediate stress granule condensation and associate with 40S subunits

(2016) Nancy Kedersha et al.   JOURNAL OF CELL BIOLOGY

Pathological Tau Promotes Neuronal Damage by Impairing Ribosomal Function and Decreasing Protein Synthesis

(2016) S. Meier et al.   JOURNAL OF NEUROSCIENCE

Alzheimer's disease

(2016) Philip Scheltens et al.   LANCET

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

In vitro prion-like behaviour of TDP-43 in ALS

(2016) Phillip Smethurst et al.   NEUROBIOLOGY OF DISEASE

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

(2016) Fernando J. Martinez et al.   NEURON

YB-1 regulates tiRNA-induced Stress Granule formation but not translational repression

(2016) Shawn M. Lyons et al.   NUCLEIC ACIDS RESEARCH

How neuroinflammation contributes to neurodegeneration

(2016) R. M. Ransohoff   SCIENCE

Vinca alkaloid drugs promote stress-induced translational repression and stress granule formation

(2016) Witold Szaflarski et al.   Oncotarget

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

(2016) Tara Vanderweyde et al.   Cell Reports

PERK inhibition prevents tau-mediated neurodegeneration in a mouse model of frontotemporal dementia

(2015) Helois Radford et al.   ACTA NEUROPATHOLOGICA

Stress granules, P-bodies and cancer

(2015) Paul Anderson et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

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

ALS-linked mutations in ubiquilin-2 or hnRNPA1 reduce interaction between ubiquilin-2 and hnRNPA1

(2015) Kathleen M. Gilpin et al.   HUMAN MOLECULAR GENETICS

Frontotemporal dementia

(2015) Jee Bang et al.   LANCET

The Human Tau Interactome: Binding to the Ribonucleoproteome, and Impaired Binding of the Proline-to-Leucine Mutant at Position 301 (P301L) to Chaperones and the Proteasome

(2015) C. Geeth Gunawardana et al.   MOLECULAR & CELLULAR PROTEOMICS

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

(2015) Yuan Lin et al.   MOLECULAR CELL

Phase Transition of a Disordered Nuage Protein Generates Environmentally Responsive Membraneless Organelles

(2015) Timothy J. Nott et al.   MOLECULAR CELL

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia

(2015) Axel Freischmidt et al.   NATURE NEUROSCIENCE

Depletion of microglia and inhibition of exosome synthesis halt tau propagation

(2015) Hirohide Asai et al.   NATURE NEUROSCIENCE

EIF4G1 mutations do not cause Parkinson's disease

(2015) Noah Nichols et al.   NEUROBIOLOGY OF AGING

Parkinson’s Disease Genes VPS35 and EIF4G1 Interact Genetically and Converge on α-Synuclein

(2015) Nripesh Dhungel et al.   NEURON

Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways

(2015) E. T. Cirulli et al.   SCIENCE

Stress Granules Modulate SYK to Cause Microglial Cell Dysfunction in Alzheimer's Disease

(2015) Soumitra Ghosh et al.   EBioMedicine

TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord

(2014) Johannes Brettschneider et al.   ACTA NEUROPATHOLOGICA

Frontotemporal lobar degeneration: defining phenotypic diversity through personalized medicine

(2014) David J. Irwin et al.   ACTA NEUROPATHOLOGICA

Phosphorylation of hnRNP K by cyclin-dependent kinase 2 controls cytosolic accumulation of TDP-43

(2014) Diane Moujalled et al.   HUMAN MOLECULAR GENETICS

Profilin 1 Associates with Stress Granules and ALS-Linked Mutations Alter Stress Granule Dynamics

(2014) M. D. Figley et al.   JOURNAL OF NEUROSCIENCE

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

(2014) David W. Sanders et al.   NEURON

Exome-wide Rare Variant Analysis Identifies TUBA4A Mutations Associated with Familial ALS

(2014) Bradley N. Smith et al.   NEURON

Stress granules are dispensable for mRNA stabilization during cellular stress

(2014) Nadine Bley et al.   NUCLEIC ACIDS RESEARCH

Free mRNA in excess upon polysome dissociation is a scaffold for protein multimerization to form stress granules

(2014) Ouissame Bounedjah et al.   NUCLEIC ACIDS RESEARCH

Ubiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: Characterization of inhibition by nucleic acids and 4-aminoquinolines

(2013) Joel A. Cassel et al.   BIOCHIMICA ET BIOPHYSICA ACTA-PROTEINS AND PROTEOMICS

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

(2013) J. Ross Buchan et al.   CELL

Amyloid-β oligomers induce synaptic damage via Tau-dependent microtubule severing by TTLL6 and spastin

(2013) Hans Zempel et al.   EMBO JOURNAL

Overexpression of ALS-Associated p.M337V Human TDP-43 in Mice Worsens Disease Features Compared to Wild-type Human TDP-43 Mice

(2013) Jonathan Janssens et al.   MOLECULAR NEUROBIOLOGY

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

(2013) Hong Joo Kim et al.   NATURE

Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models

(2013) Hyung-Jun Kim et al.   NATURE GENETICS

Suppression of eIF2α kinases alleviates Alzheimer's disease–related plasticity and memory deficits

(2013) Tao Ma et al.   NATURE NEUROSCIENCE

Unconventional Translation of C9ORF72 GGGGCC Expansion Generates Insoluble Polypeptides Specific to c9FTD/ALS

(2013) Peter E.A. Ash et al.   NEURON

U1 small nuclear ribonucleoprotein complex and RNA splicing alterations in Alzheimer's disease

(2013) B. Bai et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hydrogen peroxide induces stress granule formation independent of eIF2α phosphorylation

(2012) Mohamed M. Emara et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

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

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

Hepatitis C Virus (HCV) Induces Formation of Stress Granules Whose Proteins Regulate HCV RNA Replication and Virus Assembly and Egress

(2012) U. Garaigorta et al.   JOURNAL OF VIROLOGY

Regulated protein aggregation: stress granules and neurodegeneration

(2012) Benjamin Wolozin   Molecular Neurodegeneration

Phase transitions in the assembly of multivalent signalling proteins

(2012) Pilong Li et al.   NATURE

Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

(2012) Chi-Hong Wu et al.   NATURE

A unifying model for mTORC1-mediated regulation of mRNA translation

(2012) Carson C. Thoreen et al.   NATURE

Sustained translational repression by eIF2α-P mediates prion neurodegeneration

(2012) Julie A. Moreno et al.   NATURE

TREM2 Variants in Alzheimer's Disease

(2012) Rita Guerreiro et al.   NEW ENGLAND JOURNAL OF MEDICINE

Selenite targets eIF4E-binding protein-1 to inhibit translation initiation and induce the assembly of non-canonical stress granules

(2012) Ken Fujimura et al.   NUCLEIC ACIDS RESEARCH

De novo design of synthetic prion domains

(2012) J. A. Toombs et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43

(2012) I.-F. Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2011) Faisal Fecto   ARCHIVES OF NEUROLOGY

Local RNA Translation at the Synapse and in Disease

(2011) L. Liu-Yesucevitz et al.   JOURNAL OF NEUROSCIENCE

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

Angiogenin-Induced tRNA Fragments Inhibit Translation Initiation

(2011) Pavel Ivanov et al.   MOLECULAR CELL

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

(2011) Han-Xiang Deng et al.   NATURE

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

A quantitative study of the neuropathology of 32 sporadic and familial cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP)

(2011) R. A. Armstrong et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Soluble amyloid  -protein dimers isolated from Alzheimer cortex directly induce Tau hyperphosphorylation and neuritic degeneration

(2011) M. Jin et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TBK1 Mediates Crosstalk Between the Innate Immune Response and Autophagy

(2011) H. Weidberg et al.   Science Signaling

Biogenesis and function of nuclear bodies

(2011) Yuntao S. Mao et al.   TRENDS IN GENETICS

TDP-43 and FUS: a nuclear affair

(2011) Dorothee Dormann et al.   TRENDS IN NEUROSCIENCES

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

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

Angiogenin-induced tRNA-derived Stress-induced RNAs Promote Stress-induced Stress Granule Assembly

(2010) Mohamed M. Emara et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2010) Andrew C. Elden et al.   NATURE

The mechanism of eukaryotic translation initiation and principles of its regulation

(2010) Richard J. Jackson et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

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

Tau Reduction Prevents Aβ-Induced Defects in Axonal Transport: Fig. 1

(2010) Keith A. Vossel et al.   SCIENCE

Antitumor Activity and Mechanism of Action of the Cyclopenta[b]benzofuran, Silvestrol

(2009) Regina Cencic et al.   PLoS One

Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis

(2009) J. E. Landers et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Structure and regulation of MARK, a kinase involved in abnormal phosphorylation of Tau protein

(2008) Thomas Timm et al.   BMC NEUROSCIENCE

TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis

(2008) Vivianna M Van Deerlin et al.   LANCET NEUROLOGY

A functional RNAi screen links O-GlcNAc modification of ribosomal proteins to stress granule and processing body assembly

(2008) Takbum Ohn et al.   NATURE CELL BIOLOGY

Fragile X Syndrome: Loss of Local mRNA Regulation Alters Synaptic Development and Function

(2008) Gary J. Bassell et al.   NEURON

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE

Stress granules: the Tao of RNA triage

(2008) Paul Anderson et al.   TRENDS IN BIOCHEMICAL SCIENCES