The Interplay of RNA Binding Proteins, Oxidative Stress and Mitochondrial Dysfunction in ALS

TDP-43 aggregation induced by oxidative stress causes global mitochondrial imbalance in ALS

(2021) Xinxin Zuo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Dysfunction in nonsense-mediated decay, protein homeostasis, mitochondrial function, and brain connectivity in ALS-FUS mice with cognitive deficits

(2021) Wan Yun Ho et al.   Acta Neuropathologica Communications

ALS/FTD-associated protein FUS induces mitochondrial dysfunction by preferentially sequestering respiratory chain complex mRNAs

(2020) Yueh-Lin Tsai et al.   GENES & DEVELOPMENT

How RNA-Binding Proteins Interact with RNA: Molecules and Mechanisms

(2020) Meredith Corley et al.   MOLECULAR CELL

ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS

(2020) Jone López-Erauskin et al.   NEURON

FUS ALS-causative mutations impair FUS autoregulation and splicing factor networks through intron retention

(2020) Jack Humphrey et al.   NUCLEIC ACIDS RESEARCH

SFPQ and Tau: critical factors contributing to rapid progression of Alzheimer’s disease

(2020) Neelam Younas et al.   ACTA NEUROPATHOLOGICA

Aberrant interaction between FUS and SFPQ in neurons in a wide range of FTLD spectrum diseases

(2020) Shinsuke Ishigaki et al.   BRAIN

SFPQ is involved in regulating arsenic-induced oxidative stress by interacting with the miRNA-induced silencing complexes

(2020) Ping Guo et al.   ENVIRONMENTAL POLLUTION

Loss of Tdp-43 disrupts the axonal transcriptome of motoneurons accompanied by impaired axonal translation and mitochondria function

(2020) Michael Briese et al.   Acta Neuropathologica Communications

Paraspeckle components NONO and PSPC1 are not mislocalized from motor neuron nuclei in sporadic ALS

(2020) Giulia E Tyzack et al.   BRAIN

TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS

(2020) Chien-Hsiung Yu et al.   CELL

FUS is lost from nuclei and gained in neurites of motor neurons in a human stem cell model of VCP-related ALS

(2020) Jasmine Harley et al.   BRAIN

Detection and Quantification of Novel C‐terminal TDP‐43 Fragments in ALS‐TDP

(2020) Emily Feneberg et al.   BRAIN PATHOLOGY

Spatiotemporal Proteomic Analysis of Stress Granule Disassembly Using APEX Reveals Regulation by SUMOylation and Links to ALS Pathogenesis

(2020) Hagai Marmor-Kollet et al.   MOLECULAR CELL

Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis

(2020) Daniel Jutzi et al.   Nature Communications

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

(2019) Ze’ev Melamed et al.   NATURE 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

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

Virus-mediated delivery of antibody targeting TAR DNA-binding protein-43 mitigates associated neuropathology

(2019) Silvia Pozzi et al.   JOURNAL OF CLINICAL INVESTIGATION

Nuclear RNA foci from C9ORF72 expansion mutation form paraspeckle-like bodies

(2019) Ana Bajc Česnik et al.   JOURNAL OF CELL SCIENCE

Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10S59L/+ mouse

(2019) Emmanuelle C. Genin et al.   ACTA NEUROPATHOLOGICA

FUS pathology in ALS is linked to alterations in multiple ALS-associated proteins and rescued by drugs stimulating autophagy

(2019) Lara Marrone et al.   ACTA NEUROPATHOLOGICA

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

(2019) Rudolf C Hergesheimer et al.   BRAIN

Mechanisms and roles of mitophagy in neurodegenerative diseases

(2019) Yan Wang et al.   CNS Neuroscience & Therapeutics

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

(2019) Peipei Zhang et al.   eLife

The Pathobiology of TDP-43 C-Terminal Fragments in ALS and FTLD

(2019) Britt A. Berning et al.   Frontiers in Neuroscience

A small molecule targeting TDP-43’s RNA recognition motifs reduces locomotor defects in a Drosophila model of ALS

(2019) Liberty Francois-Moutal et al.   ACS Chemical Biology

Widespread FUS mislocalization is a molecular hallmark of amyotrophic lateral sclerosis

(2019) Giulia E Tyzack et al.   BRAIN

Small-Molecule Modulation of TDP-43 Recruitment to Stress Granules Prevents Persistent TDP-43 Accumulation in ALS/FTD

(2019) Mark Y. Fang et al.   NEURON

Mutant FUS and ELAVL4 (HuD) Aberrant Crosstalk in Amyotrophic Lateral Sclerosis

(2019) Riccardo De Santis et al.   Cell Reports

Stress granules regulate stress-induced paraspeckle assembly

(2019) Haiyan An et al.   JOURNAL OF CELL BIOLOGY

TDP-43 proteinopathy and mitochondrial abnormalities in neurodegeneration

(2019) Ju Gao et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

A role for SUMOylation in the Formation and Cellular Localization of TDP-43 Aggregates in Amyotrophic Lateral Sclerosis

(2019) Cindy Maurel et al.   MOLECULAR NEUROBIOLOGY

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

(2018) Mario Hofweber et al.   CELL

Respiratory Failure in Amyotrophic Lateral Sclerosis

(2018) Shannon Niedermeyer et al.   CHEST

Mice with endogenous TDP‐43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis

(2018) Pietro Fratta et al.   EMBO JOURNAL

TDP-43 post-translational modifications in health and disease

(2018) Emanuele Buratti   EXPERT OPINION ON THERAPEUTIC TARGETS

The Role of Post-Translational Modifications on Prion-Like Aggregation and Liquid-Phase Separation of FUS

(2018) Shannon Rhoads et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Differential toxicity of TDP-43 isoforms depends on their sub-mitochondrial localization in neuronal cells

(2018) Illari Salvatori et al.   JOURNAL OF NEUROCHEMISTRY

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

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

A brave new world of RNA-binding proteins

(2018) Matthias W. Hentze et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics

(2018) Stephani A. Davis et al.   NEUROSCIENCE LETTERS

Paraspeckles: Where Long Noncoding RNA Meets Phase Separation

(2018) Archa H. Fox et al.   TRENDS IN BIOCHEMICAL SCIENCES

Intron retention and nuclear loss of SFPQ are molecular hallmarks of ALS

(2018) Raphaelle Luisier et al.   Nature Communications

Riluzole and edaravone: A tale of two amyotrophic lateral sclerosis drugs

(2018) Manoj Kumar Jaiswal   MEDICINAL RESEARCH REVIEWS

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

Ubiquilin 2 modulates ALS/FTD-linked FUS–RNA complex dynamics and stress granule formation

(2018) Elizabeth J. Alexander et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice

(2017) Eva So et al.   HUMAN MOLECULAR GENETICS

Methylarginines within the RGG-Motif Region of hnRNP A1 Affect Its IRES Trans -Acting Factor Activity and Are Required for hnRNP A1 Stress Granule Localization and Formation

(2017) Michael L. Wall et al.   JOURNAL OF MOLECULAR BIOLOGY

Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo

(2017) Hibiki Kawamata et al.   Molecular Neurodegeneration

ALS-Related Mutant FUS Protein Is Mislocalized to Cytoplasm and Is Recruited into Stress Granules of Fibroblasts from Asymptomatic FUS P525L Mutation Carriers

(2017) Margherita Lo Bello et al.   Neurodegenerative Diseases

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

Acetylation-induced TDP-43 pathology is suppressed by an HSF1-dependent chaperone program

(2017) Ping Wang et al.   Nature Communications

Loss of function CHCHD10 mutations in cytoplasmic TDP-43 accumulation and synaptic integrity

(2017) Jung-A. A. Woo et al.   Nature Communications

Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS

(2017) Claire E. Hall et al.   Cell Reports

Clinical and neuropathological features of ALS/FTD with TIA1 mutations

(2017) Veronica Hirsch-Reinshagen et al.   Acta Neuropathologica Communications

TDP-43 stabilises the processing intermediates of mitochondrial transcripts

(2017) Keiichi Izumikawa et al.   Scientific Reports

ALS Associated Mutations in Matrin 3 Alter Protein-Protein Interactions and Impede mRNA Nuclear Export

(2017) Ashley Boehringer et al.   Scientific Reports

Inside out: the role of nucleocytoplasmic transport in ALS and FTLD

(2016) Steven Boeynaems et al.   ACTA NEUROPATHOLOGICA

Imbalance of mitochondrial dynamics in Drosophila models of amyotrophic lateral sclerosis

(2016) Volodya Altanbyek et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

T-cell-restricted intracellular antigen 1 facilitates mitochondrial fragmentation by enhancing the expression of mitochondrial fission factor

(2016) Hyosun Tak et al.   CELL DEATH AND DIFFERENTIATION

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity

(2016) Wenzhang Wang et al.   NATURE MEDICINE

The RNA-binding protein SFPQ orchestrates an RNA regulon to promote axon viability

(2016) Katharina E Cosker et al.   NATURE NEUROSCIENCE

Gene discovery in amyotrophic lateral sclerosis: implications for clinical management

(2016) Ammar Al-Chalabi et al.   Nature Reviews Neurology

Poly(GR) in C9ORF72 -Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons

(2016) Rodrigo Lopez-Gonzalez et al.   NEURON

Super-Resolution Microscopy Reveals Presynaptic Localization of the ALS/FTD Related Protein FUS in Hippocampal Neurons

(2016) Michael Schoen et al.   Frontiers in Cellular Neuroscience

Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses

(2016) Katannya Kapeli et al.   Nature Communications

TIA1 oxidation inhibits stress granule assembly and sensitizes cells to stress-induced apoptosis

(2016) Kyoko Arimoto-Matsuzaki et al.   Nature Communications

ALS-Causing Mutations Significantly Perturb the Self-Assembly and Interaction with Nucleic Acid of the Intrinsically Disordered Prion-Like Domain of TDP-43

(2016) Liangzhong Lim et al.   PLOS BIOLOGY

Heterogeneous nuclear ribonucleoprotein A1 post-transcriptionally regulates Drp1 expression in neuroblastoma cells

(2015) So Jung Park et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

(2015) Avinash Patel et al.   CELL

ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cell-derived motoneurons

(2015) J. Lenzi et al.   Disease Models & Mechanisms

PRMT1-mediated arginine methylation controls ATXN2L localization

(2015) Christian Kaehler et al.   EXPERIMENTAL CELL RESEARCH

Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations

(2015) M. J. Finelli et al.   HUMAN MOLECULAR GENETICS

Frontotemporal dementia

(2015) Jee Bang et al.   LANCET

Inactivation of EWS reduces PGC-1α protein stability and mitochondrial homeostasis

(2015) Jun Hong Park et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD

(2015) J. P. Ling et al.   SCIENCE

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

The cleavage pattern of TDP-43 determines its rate of clearance and cytotoxicity

(2015) Quan Li et al.   Nature Communications

An acetylation switch controls TDP-43 function and aggregation propensity

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

FUS Interacts with HSP60 to Promote Mitochondrial Damage

(2015) Jianwen Deng et al.   PLoS Genetics

C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis

(2015) Johnathan Cooper-Knock et al.   PLoS One

Amyotrophic lateral sclerosis onset is influenced by the burden of rare variants in known amyotrophic lateral sclerosis genes

(2014) Janet Cady et al.   ANNALS OF NEUROLOGY

Methyl pyruvate rescues mitochondrial damage caused by SIGMAR1 mutation related to amyotrophic lateral sclerosis

(2014) Hideaki Tagashira et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Mutations in the CHCHD10 gene are a common cause of familial amyotrophic lateral sclerosis

(2014) Janel O. Johnson et al.   BRAIN

Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions

(2014) Johnathan Cooper-Knock et al.   BRAIN

A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement

(2014) Sylvie Bannwarth et al.   BRAIN

Oxidative stress affects FET proteins localization and alternative pre-mRNA processing in cellular models of ALS

(2014) Francesca Svetoni et al.   FREE RADICAL BIOLOGY AND MEDICINE

ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects

(2014) Haiyan Qiu et al.   JOURNAL OF CLINICAL INVESTIGATION

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

(2014) Janel O Johnson et al.   NATURE NEUROSCIENCE

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

(2014) Nael H. Alami et al.   NEURON

Loss of hnRNPA1 in ALS spinal cord motor neurons with TDP-43-positive inclusions

(2014) Hiroyuki Honda et al.   NEUROPATHOLOGY

ER–mitochondria associations are regulated by the VAPB–PTPIP51 interaction and are disrupted by ALS/FTD-associated TDP-43

(2014) Radu Stoica et al.   Nature Communications

Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS

(2013) Tania F. Gendron et al.   ACTA NEUROPATHOLOGICA

Molecular mechanism of oxidation-induced TDP-43 RRM1 aggregation and loss of function

(2013) Chung-ke Chang et al.   FEBS LETTERS

Clinical perspective on oxidative stress in sporadic amyotrophic lateral sclerosis

(2013) Emanuele D’Amico et al.   FREE RADICAL BIOLOGY AND MEDICINE

Compromised paraspeckle formation as a pathogenic factor in FUSopathies

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

The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons

(2013) Wenzhang Wang et al.   HUMAN MOLECULAR GENETICS

FUS/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress

(2013) Reddy Ranjith K. Sama et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Dexpramipexole versus placebo for patients with amyotrophic lateral sclerosis (EMPOWER): a randomised, double-blind, phase 3 trial

(2013) Merit E Cudkowicz et al.   LANCET NEUROLOGY

Amyotrophic lateral sclerosis-linked FUS/TLS alters stress granule assembly and dynamics

(2013) Desiree M Baron et al.   Molecular Neurodegeneration

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

(2013) Hong Joo Kim et al.   NATURE

T-cell Intracellular Antigen (TIA)-Proteins Deficiency in Murine Embryonic Fibroblasts Alters Cell Cycle Progression and Induces Autophagy

(2013) Carmen Sánchez-Jiménez et al.   PLoS One

FUS regulates genes coding for RNA-binding proteins in neurons by binding to their highly conserved introns

(2013) T. Nakaya et al.   RNA

FUS-regulated region- and cell-type-specific transcriptome is associated with cell selectivity in ALS/FTLD

(2013) Yusuke Fujioka et al.   Scientific 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

The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons

(2013) Daiyu Honda et al.   FEBS Open Bio

TDP-43 aggregation in neurodegeneration: Are stress granules the key?

(2012) Colleen M. Dewey et al.   BRAIN RESEARCH

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

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

Mitochondrial dysfunction in human TDP-43 transfected NSC34 cell lines and the protective effect of dimethoxy curcumin

(2012) Jinsheng Lu et al.   BRAIN RESEARCH BULLETIN

Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS

(2012) Dorothee Dormann et al.   EMBO JOURNAL

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

(2012) Takao Naganuma et al.   EMBO JOURNAL

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

(2012) Julien Couthouis et al.   HUMAN MOLECULAR GENETICS

RNA Binding Proteins Accumulate at the Postsynaptic Density with Synaptic Activity

(2012) G. Zhang et al.   JOURNAL OF NEUROSCIENCE

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs

(2012) Clotilde Lagier-Tourenne et al.   NATURE NEUROSCIENCE

Riluzole-Triggered GSH Synthesis via Activation of Glutamate Transporters to Antagonize Methylmercury-Induced Oxidative Stress in Rat Cerebral Cortex

(2012) Yu Deng et al.   Oxidative Medicine and Cellular Longevity

Ataxin-2-Like Is a Regulator of Stress Granules and Processing Bodies

(2012) Christian Kaehler et al.   PLoS One

Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice

(2012) H Z Yin et al.   Cell Death & Disease

Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions

(2012) Shinsuke Ishigaki et al.   Scientific Reports

Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain

(2012) Boris Rogelj et al.   Scientific Reports

Mutational analysis reveals the FUS homolog TAF15 as a candidate gene for familial amyotrophic lateral sclerosis

(2011) N. Ticozzi et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART B-NEUROPSYCHIATRIC GENETICS

Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linking

(2011) Todd J Cohen et al.   EMBO JOURNAL

TDP-43-induced Death Is Associated with Altered Regulation of BIM and Bcl-xL and Attenuated by Caspase-mediated TDP-43 Cleavage

(2011) Hiroaki Suzuki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study

(2011) Julie Phukan et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress

(2011) Jodi Meyerowitz et al.   Molecular Neurodegeneration

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43

(2011) Magdalini Polymenidou et al.   NATURE NEUROSCIENCE

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

(2011) James R Tollervey et al.   NATURE NEUROSCIENCE

Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies

(2011) Yohei Iguchi et al.   NEUROBIOLOGY OF DISEASE

Hyperphosphorylation as a Defense Mechanism to Reduce TDP-43 Aggregation

(2011) Huei-Ying Li et al.   PLoS One

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

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

(2010) Youhna M Ayala et al.   EMBO JOURNAL

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

(2010) Dorothee Dormann et al.   EMBO JOURNAL

Regulation of TDP-43 aggregation by phosphorylation andp62/SQSTM1

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

Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis

(2010) S. J. Barmada et al.   JOURNAL OF NEUROSCIENCE

Wild-Type Human TDP-43 Expression Causes TDP-43 Phosphorylation, Mitochondrial Aggregation, Motor Deficits, and Early Mortality in Transgenic Mice

(2010) Y.-F. Xu et al.   JOURNAL OF NEUROSCIENCE

Nuclear localization sequence of FUS and induction of stress granules by ALS mutants

(2010) Jozsef Gal et al.   NEUROBIOLOGY OF AGING

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

Phase II trial of CoQ10 for ALS finds insufficient evidence to justify phase III

(2009) Petra Kaufmann et al.   ANNALS OF NEUROLOGY

Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

(2009) Siân C. Barber et al.   FREE RADICAL BIOLOGY AND MEDICINE

Expression of TDP-43 C-terminal Fragmentsin VitroRecapitulates Pathological Features of TDP-43 Proteinopathies

(2009) Lionel M. Igaz 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

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity

(2009) Y.-J. Zhang 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

Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

(2008) Masato Hasegawa et al.   ANNALS OF NEUROLOGY

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE