The implications of physiological biomolecular condensates in amyotrophic lateral sclerosis

lncRNA NEAT1: key player in neurodegenerative diseases

(2023) Kun Li et al.   AGEING RESEARCH REVIEWS

Regulation of ribosomal RNA gene copy number, transcription and nucleolus organization in eukaryotes

(2023) Yutaro Hori et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

FUS regulates a subset of snoRNA expression and modulates the level of rRNA modifications

(2023) Kishor Gawade et al.   Scientific Reports

Liquid-Liquid Phase Separation of TDP-43 and FUS in Physiology and Pathology of Neurodegenerative Diseases

(2022) Jenny L. Carey et al.   Frontiers in Molecular Biosciences

Condensates induced by transcription inhibition localize active chromatin to nucleoli

(2022) Takaaki Yasuhara et al.   MOLECULAR CELL

Dysregulation of Translation in TDP-43 Proteinopathies: Deficits in the RNA Supply Chain and Local Protein Production

(2022) Reed T. Bjork et al.   Frontiers in Neuroscience

Sequence Determinants of TDP-43 Ribonucleoprotein Condensate Formation and Axonal Transport in Neurons

(2022) Sonali S. Vishal et al.   Frontiers in Cell and Developmental Biology

Stress Granule Homeostasis, Aberrant Phase Transition, and Amyotrophic Lateral Sclerosis

(2022) Zhanxu Li et al.   ACS Chemical Neuroscience

Stress granules in the spinal muscular atrophy and amyotrophic lateral sclerosis: The correlation and promising therapy

(2022) LiDan Hu et al.   NEUROBIOLOGY OF DISEASE

Nuclear RNA binding regulates TDP-43 nuclear localization and passive nuclear export

(2022) Lauren Duan et al.   Cell Reports

Hyperosmotic-stress-induced liquid-liquid phase separation of ALS-related proteins in the nucleus

(2022) Chao Gao et al.   Cell Reports

Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS

(2022) Timothy M. Miller et al.   NEW ENGLAND JOURNAL OF MEDICINE

Familial ALS-associated SFPQ variants promote the formation of SFPQ cytoplasmic aggregates in primary neurons

(2022) Jocelyn Widagdo et al.   Open Biology

Long non-coding RNA Neat1 and paraspeckle components are translational regulators in hypoxia

(2022) Anne-Claire Godet et al.   eLife

FUS and TDP-43 Phases in Health and Disease

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

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

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

Nucleolin Rescues TDP-43 Toxicity in Yeast and Human Cell Models

(2021) Caterina Peggion et al.   Frontiers in Cellular Neuroscience

Connecting the “dots”: RNP granule network in health and disease

(2021) Haiyan An et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

It’s not just a phase: function and characteristics of RNA-binding proteins in phase separation

(2021) Hannah J. Wiedner et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

TOR signaling regulates liquid phase separation of the SMN complex governing snRNP biogenesis

(2021) Maximilian Schilling et al.   Cell Reports

FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translation

(2021) Nicol Birsa et al.   Science Advances

Aberrant Stress Granule Dynamics and Aggrephagy in ALS Pathogenesis

(2021) Yi Zhang et al.   Cells

Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins

(2021) Topaz Altman et al.   Nature Communications

ALS-derived fibroblasts exhibit reduced proliferation rate, cytoplasmic TDP-43 aggregation and a higher susceptibility to DNA damage

(2020) Javier Riancho et al.   JOURNAL OF NEUROLOGY

Characteristic Features of FUS Inclusions in Spinal Motor Neurons of Sporadic Amyotrophic Lateral Sclerosis

(2020) Kensuke Ikenaka et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

The Overlapping Genetics of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

(2020) Yevgeniya A. Abramzon et al.   Frontiers in Neuroscience

Molecular dissection of ALS‐linked TDP‐43 – involvement of the Gly‐rich domain in interaction with G‐quadruplex mRNA

(2020) Akira Ishiguro et al.   FEBS LETTERS

Formation of liquid-like cellular organelles depends on their composition

(2020) Chiu Fan Lee   NATURE

Composition-dependent thermodynamics of intracellular phase separation

(2020) Joshua A. Riback et al.   NATURE

UsnRNP trafficking is regulated by stress granules and compromised by mutant ALS proteins

(2020) Simona Rossi et al.   NEUROBIOLOGY OF DISEASE

Dysregulation of RNA-Binding Proteins in Amyotrophic Lateral Sclerosis

(2020) Yuan Chao Xue et al.   Frontiers in Molecular Neuroscience

Opportunities for Histone Deacetylase Inhibition in Amyotrophic Lateral Sclerosis

(2020) Yvonne E. Klingl et al.   BRITISH JOURNAL OF PHARMACOLOGY

Stress Induces Dynamic, Cytotoxicity-Antagonizing TDP-43 Nuclear Bodies via Paraspeckle LncRNA NEAT1-Mediated Liquid-Liquid Phase Separation

(2020) Chen Wang et al.   MOLECULAR CELL

TDP-43 transports ribosomal protein mRNA to regulate axonal local translation in neuronal axons

(2020) Seiichi Nagano et al.   ACTA NEUROPATHOLOGICA

The role of TDP-43 mislocalization in amyotrophic lateral sclerosis

(2020) Terry R. Suk et al.   Molecular Neurodegeneration

The nucleolus as a multiphase liquid condensate

(2020) Denis L. J. Lafontaine et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Walking the line: mechanisms underlying directional mRNA transport and localisation in neurons and beyond

(2020) Reem Abouward et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

The Neuromuscular Junction in Health and Disease: Molecular Mechanisms Governing Synaptic Formation and Homeostasis

(2020) Pedro M. Rodríguez Cruz et al.   Frontiers in Molecular Neuroscience

ALS-linked FUS mutations confer loss and gain of function in the nucleus by promoting excessive formation of dysfunctional paraspeckles

(2019) Haiyan An et al.   Acta Neuropathologica Communications

TDP-43 regulates site-specific 2′-O-methylation of U1 and U2 snRNAs via controlling the Cajal body localization of a subset of C/D scaRNAs

(2019) Keiichi Izumikawa et al.   NUCLEIC ACIDS RESEARCH

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 debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?

(2019) Rudolf C Hergesheimer et al.   BRAIN

Cross-Regulation between TDP-43 and Paraspeckles Promotes Pluripotency-Differentiation Transition

(2019) Miha Modic et al.   MOLECULAR CELL

Widespread FUS mislocalization is a molecular hallmark of amyotrophic lateral sclerosis

(2019) Giulia E Tyzack et al.   BRAIN

Local translation in neurons: visualization and function

(2019) Christine E. Holt et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

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

(2019) F. Frottin et al.   SCIENCE

RNA Granules Hitchhike on Lysosomes for Long-Distance Transport, Using Annexin A11 as a Molecular Tether

(2019) Ya-Cheng Liao et al.   CELL

Stress granules and neurodegeneration

(2019) Benjamin Wolozin et al.   NATURE REVIEWS NEUROSCIENCE

TDP-43 Regulates Coupled Dendritic mRNA Transport-Translation Processes in Co-operation with FMRP and Staufen1

(2019) Jen-Fei Chu et al.   Cell Reports

ALS Genetics, Mechanisms, and Therapeutics: Where Are We Now?

(2019) Rita Mejzini et al.   Frontiers in Neuroscience

Transcriptome–pathology correlation identifies interplay between TDP-43 and the expression of its kinase CK1E in sporadic ALS

(2018) Florian Krach et al.   ACTA NEUROPATHOLOGICA

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

(2018) Jie Wang 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

RGG/RG Motif Regions in RNA Binding and Phase Separation

(2018) P. Andrew Chong et al.   JOURNAL OF MOLECULAR BIOLOGY

Functional Domains of NEAT1 Architectural lncRNA Induce Paraspeckle Assembly through Phase Separation

(2018) Tomohiro Yamazaki et al.   MOLECULAR CELL

Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis

(2018) Tatyana A. Shelkovnikova et al.   Molecular Neurodegeneration

Self-interaction of NPM1 modulates multiple mechanisms of liquid–liquid phase separation

(2018) Diana M. Mitrea et al.   Nature Communications

A complex of C9ORF72 and p62 uses arginine methylation to eliminate stress granules by autophagy

(2018) Maneka Chitiprolu et al.   Nature Communications

Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization

(2018) Emile S. Pinarbasi et al.   Scientific Reports

Nuclear egress of TDP-43 and FUS occurs independently of Exportin-1/CRM1

(2018) Helena Ederle et al.   Scientific Reports

Axonal G3BP1 stress granule protein limits axonal mRNA translation and nerve regeneration

(2018) Pabitra K. Sahoo et al.   Nature Communications

TDP-43 and FUS en route from the nucleus to the cytoplasm

(2017) Helena Ederle et al.   FEBS LETTERS

Heat Shock-induced Phosphorylation of TAR DNA-binding Protein 43 (TDP-43) by MAPK/ERK Kinase Regulates TDP-43 Function

(2017) Wen Li et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Point mutations in the N-terminal domain of transactive response DNA-binding protein 43 kDa (TDP-43) compromise its stability, dimerization, and functions

(2017) Miguel Mompeán et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

New perspectives of physiological and pathological functions of nucleolin (NCL)

(2017) Wenyu Jia et al.   LIFE SCIENCES

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

(2017) Lindsay A. Becker et al.   NATURE

NEAT1 scaffolds RNA-binding proteins and the Microprocessor to globally enhance pri-miRNA processing

(2017) Li Jiang et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Non-nuclear Pool of Splicing Factor SFPQ Regulates Axonal Transcripts Required for Normal Motor Development

(2017) Swapna Thomas-Jinu et al.   NEURON

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

(2017) Ian R. Mackenzie et al.   NEURON

Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons

(2017) Pallavi P. Gopal 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

HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients

(2017) Wenting Guo et al.   Nature Communications

NEAT1 scaffolds RNA-binding proteins and the Microprocessor to globally enhance pri-miRNA processing

(2017) Li Jiang et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Minor intron splicing is regulated by FUS and affected by ALS‐associated FUS mutants

(2016) Stefan Reber et al.   EMBO JOURNAL

TDP-43 binds and transports G-quadruplex-containing mRNAs into neurites for local translation

(2016) Akira Ishiguro et al.   GENES TO CELLS

Structural, super-resolution microscopy analysis of paraspeckle nuclear body organization

(2016) Jason A. West et al.   JOURNAL OF CELL BIOLOGY

Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins

(2016) Antonia Ratti et al.   JOURNAL OF NEUROCHEMISTRY

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

Distinct stages in stress granule assembly and disassembly

(2016) Joshua R Wheeler et al.   eLife

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

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

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

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

Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS?

(2015) Anaïs Aulas et al.   Frontiers in Cellular Neuroscience

An acetylation switch controls TDP-43 function and aggregation propensity

(2015) Todd J. Cohen et al.   Nature Communications

ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP

(2015) Shuying Sun et al.   Nature Communications

FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis

(2015) Yoshihiro Kino et al.   Acta Neuropathologica Communications

Transcriptomic Changes Due to Cytoplasmic TDP-43 Expression Reveal Dysregulation of Histone Transcripts and Nuclear Chromatin

(2015) Alexandre Amlie-Wolf et al.   PLoS One

Liquid-Liquid Phase Separation in Biology

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

Gem Depletion: Amyotrophic Lateral Sclerosis and Spinal Muscular Atrophy Crossover

(2014) Ruben J. Cauchi   CNS Neuroscience & Therapeutics

The Mechanistic Links Between Proteasome Activity, Aging and Agerelated Diseases

(2014) Isabel Saez et al.   CURRENT GENOMICS

ALS-Linked Mutations Enlarge TDP-43-Enriched Neuronal RNA Granules in the Dendritic Arbor

(2014) L. Liu-Yesucevitz et al.   JOURNAL OF NEUROSCIENCE

Long Noncoding RNA NEAT1-Dependent SFPQ Relocation from Promoter Region to Paraspeckle Mediates IL8 Expression upon Immune Stimuli

(2014) Katsutoshi Imamura et al.   MOLECULAR CELL

Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations

(2014) Nael H. Alami et al.   NEURON

Nuclear localization of SMN and FUS is not altered in fibroblasts from patients with sporadic ALS

(2014) Shingo Kariya et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Protein aggregation in amyotrophic lateral sclerosis

(2013) Anna M. Blokhuis et al.   ACTA NEUROPATHOLOGICA

Reactive nucleolar and Cajal body responses to proteasome inhibition in sensory ganglion neurons

(2013) Ana Palanca et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

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

(2013) J. Ross Buchan et al.   CELL

Compromised paraspeckle formation as a pathogenic factor in FUSopathies

(2013) Tatyana A. Shelkovnikova et al.   HUMAN MOLECULAR GENETICS

NEAT1 long noncoding RNA regulates transcription via protein sequestration within subnuclear bodies

(2013) Tetsuro Hirose et al.   MOLECULAR BIOLOGY OF THE CELL

Defining the RGG/RG Motif

(2013) Palaniraja Thandapani et al.   MOLECULAR CELL

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

(2013) Shuo-Chien Ling et al.   NEURON

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

Alternative 3′-end processing of long noncoding RNA initiates construction of nuclear paraspeckles

(2012) Takao Naganuma et al.   EMBO JOURNAL

Spliceosome integrity is defective in the motor neuron diseases ALS and SMA

(2012) Hitomi Tsuiji et al.   EMBO Molecular Medicine

TDP-43 promotes microRNA biogenesis as a component of the Drosha and Dicer complexes

(2012) Y. Kawahara et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA

(2012) Tomohiro Yamazaki et al.   Cell Reports

Characterizing the RNA targets and position-dependent splicing regulation by TDP-43

(2011) James R Tollervey et al.   NATURE NEUROSCIENCE

RNA targets of wild-type and mutant FET family proteins

(2011) Jessica I Hoell et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

FUS/TLS forms cytoplasmic aggregates, inhibits cell growth and interacts with TDP-43 in a yeast model of amyotrophic lateral sclerosis

(2011) Dmitry Kryndushkin et al.   Protein & Cell

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

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

Identification of Neuronal RNA Targets of TDP-43-containing Ribonucleoprotein Complexes

(2010) Chantelle F. Sephton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice

(2010) X. Shan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Age-Dependent Deterioration of Nuclear Pore Complexes Causes a Loss of Nuclear Integrity in Postmitotic Cells

(2009) Maximiliano A. D'Angelo et al.   CELL

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

TDP-43 is recruited to stress granules in conditions of oxidative insult

(2009) Claudia Colombrita et al.   JOURNAL OF NEUROCHEMISTRY

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

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

An Architectural Role for a Nuclear Noncoding RNA: NEAT1 RNA Is Essential for the Structure of Paraspeckles

(2009) Christine M. Clemson et al.   MOLECULAR CELL

Altered Nuclear Retention of mRNAs Containing Inverted Repeats in Human Embryonic Stem Cells: Functional Role of a Nuclear Noncoding RNA

(2009) Ling-Ling Chen et al.   MOLECULAR CELL

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

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

Structural determinants of the cellular localization and shuttling of TDP-43

(2008) Y. M. Ayala et al.   JOURNAL OF CELL SCIENCE