相关参考文献
注意:仅列出部分参考文献,下载原文获取全部文献信息。Axonal transport defects and neurodegeneration: Molecular mechanisms and therapeutic implications
Wenting Guo et al.
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY (2020)
Symmetric dimethylation of poly-GR correlates with disease duration in C9orf72 FTLD and ALS and reduces poly-GR phase separation and toxicity
Lauren M. Gittings et al.
ACTA NEUROPATHOLOGICA (2020)
Competing Protein-RNA Interaction Networks Control Multiphase Intracellular Organization
David W. Sanders et al.
CELL (2020)
G3BP1 Is a Tunable Switch that Triggers Phase Separation to Assemble Stress Granules
Peiguo Yang et al.
CELL (2020)
RNA-Induced Conformational Switching and Clustering of G3BP Drive Stress Granule Assembly by Condensation
Jordina Guillen-Boixet et al.
CELL (2020)
Hsp27 chaperones FUS phase separation under the modulation of stress-induced phosphorylation
Zhenying Liu et al.
NATURE STRUCTURAL & MOLECULAR BIOLOGY (2020)
The Loss of TBK1 Kinase Activity in Motor Neurons or in All Cell Types Differentially Impacts ALS Disease Progression in SOD1 Mice
Valeria Gerbino et al.
NEURON (2020)
The role of mitochondria in amyotrophic lateral sclerosis
Emma F. Smith et al.
NEUROSCIENCE LETTERS (2019)
RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43
Jacob R. Mann et al.
NEURON (2019)
Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death
Fatima Gasset-Rosa et al.
NEURON (2019)
Purification of cross-linked RNA-protein complexes by phenol-toluol extraction
Erika C. Urdaneta et al.
NATURE COMMUNICATIONS (2019)
FUS pathology in ALS is linked to alterations in multiple ALS-associated proteins and rescued by drugs stimulating autophagy
Lara Marrone et al.
ACTA NEUROPATHOLOGICA (2019)
ADAR2 mislocalization and widespread RNA editing aberrations in C9orf72-mediated ALS/FTD
Stephen Moore et al.
ACTA NEUROPATHOLOGICA (2019)
Computational identification of prion-like RNA-binding proteins that form liquid phase-separated condensates
Gabriele Orlando et al.
BIOINFORMATICS (2019)
Yeast Ataxin-2 Forms an Intracellular Condensate Required for the Inhibition of TORC1 Signaling during Respiratory Growth
Yu-San Yang et al.
CELL (2019)
Redox State Controls Phase Separation of the Yeast Ataxin-2 Protein via Reversible Oxidation of Its Methionine-Rich Low-Complexity Domain
Masato Kato et al.
CELL (2019)
PARP-1 Activation Directs FUS to DNA Damage Sites to Form PARG-Reversible Compartments Enriched in Damaged DNA
Anastasia S. Singatulina et al.
CELL REPORTS (2019)
Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology
Peipei Zhang et al.
ELIFE (2019)
Widespread FUS mislocalization is a molecular hallmark of amyotrophic lateral sclerosis
Giulia E. Tyzack et al.
BRAIN (2019)
Epidemiology of amyotrophic lateral sclerosis: an update of recent literature
Elisa Longinetti et al.
CURRENT OPINION IN NEUROLOGY (2019)
Covalent targeting of the vacuolar H+-ATPase activates autophagy via mTORC1 inhibition
Clive Yik-Sham Chung et al.
NATURE CHEMICAL BIOLOGY (2019)
Small-Molecule Modulation of TDP-43 Recruitment to Stress Granules Prevents Persistent TDP-43 Accumulation in ALS/FTD
Mark Y. Fang et al.
NEURON (2019)
Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia
Laurence Renaud et al.
ACTA NEUROPATHOLOGICA COMMUNICATIONS (2019)
RNA Granules Hitchhike on Lysosomes for Long-Distance Transport, Using Annexin A11 as a Molecular Tether
Ya-Cheng Liao et al.
CELL (2019)
Properties of Stress Granule and P-Body Proteomes
Ji-Young Youn et al.
MOLECULAR CELL (2019)
Exome sequencing in amyotrophic lateral sclerosis implicates a novel gene, DNAJC7, encoding a heat-shock protein
Sali M. K. Farhan et al.
NATURE NEUROSCIENCE (2019)
The mutational landscape of a prion-like domain
Benedetta Bolognesi et al.
NATURE COMMUNICATIONS (2019)
C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport
Joni Vanneste et al.
SCIENTIFIC REPORTS (2019)
The Human RNA-Binding Proteome and Its Dynamics during Translational Arrest
Jakob Trendel et al.
CELL (2019)
ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair
Joseph R. Klim et al.
NATURE NEUROSCIENCE (2019)
Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration
Ze'ev Melamed et al.
NATURE NEUROSCIENCE (2019)
RNA Dysregulation in Amyotrophic Lateral Sclerosis
Zoe Butti et al.
FRONTIERS IN GENETICS (2019)
Disrupted neuronal trafficking in amyotrophic lateral sclerosis
Katja Burk et al.
ACTA NEUROPATHOLOGICA (2019)
Reconsidering the causality of TIA1 mutations in ALS
Rick A. Van Der Spek et al.
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION (2018)
Matrin 3 Is a Component of Neuronal Cytoplasmic Inclusions of Motor Neurons in Sporadic Amyotrophic Lateral Sclerosis
Mikiko Tada et al.
AMERICAN JOURNAL OF PATHOLOGY (2018)
Hot-spot KIF5A mutations cause familial ALS
David Brenner et al.
BRAIN (2018)
Nuclear Import Receptor Inhibits Phase Separation of FUS through Binding to Multiple Sites
Takuya Yoshizawa et al.
CELL (2018)
Phase Separation of FUS Is Suppressed by Its Nuclear Import Receptor and Arginine Methylation
Mario Hofweber et al.
CELL (2018)
Context-Dependent and Disease-Specific Diversity in Protein Interactions within Stress Granules
Sebastian Markmiller et al.
CELL (2018)
Stress Granule Assembly Disrupts Nucleocytoplasmic Transport
Ke Zhang et al.
CELL (2018)
Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains
Lin Guo et al.
CELL (2018)
FUS Phase Separation Is Modulated by a Molecular Chaperone and Methylation of Arginine Cation-π Interactions
Seema Qamar et al.
CELL (2018)
Polyubiquitin chain-induced p62 phase separation drives autophagic cargo segregation
Daxiao Sun et al.
CELL RESEARCH (2018)
Karyopherins in cancer
Tolga Cagatay et al.
CURRENT OPINION IN CELL BIOLOGY (2018)
Ubiquitin Modulates Liquid-Liquid Phase Separation of UBQLN2 via Disruption of Multivalent Interactions
Thuy P. Dao et al.
MOLECULAR CELL (2018)
Mechanistic View of hnRNPA2 Low-Complexity Domain Structure, Interactions, and Phase Separation Altered by Mutation and Arginine Methylation
Veronica H. Ryan et al.
MOLECULAR CELL (2018)
DNA plasticity and damage in amyotrophic lateral sclerosis
Alexandra Kretz et al.
Neural Regeneration Research (2018)
RNP-Granule Assembly via Ataxin-2 Disordered Domains Is Required for Long-Term Memory and Neurodegeneration
Baskar Bakthavachalu et al.
NEURON (2018)
Genome-wide Analyses Identify KIF5A as a Novel ALS Gene
Aude Nicolas et al.
NEURON (2018)
RNA buffers the phase separation behavior of prion-like RNA binding proteins
Shovamayee Maharana et al.
SCIENCE (2018)
NEK1 loss-of-function mutation induces DNA damage accumulation in ALS patient-derived motoneurons
Julia Higelin et al.
STEM CELL RESEARCH (2018)
ALS Genes in the Genomic Era and their Implications for FTD
Hung Phuoc Nguyen et al.
TRENDS IN GENETICS (2018)
Molecular Dissection of FUS Points at Synergistic Effect of Low-Complexity Domains in Toxicity
Elke Bogaert et al.
CELL REPORTS (2018)
Modeling Protein Aggregation and the Heat Shock Response in ALS iPSC-Derived Motor Neurons
Emily R. Seminary et al.
FRONTIERS IN NEUROSCIENCE (2018)
Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization
Leeanne McGurk et al.
MOLECULAR CELL (2018)
Kinase-controlled phase transition of membraneless organelles in mitosis
Arpan Kumar Rai et al.
NATURE (2018)
C9orf72-mediated ALS and FTD: multiple pathways to disease
Rubika Balendra et al.
NATURE REVIEWS NEUROLOGY (2018)
C9orf72, a protein associated with amyotrophic lateral sclerosis (ALS) is a guanine nucleotide exchange factor
Shalini Iyer et al.
PEERJ (2018)
Effects of Mutations on the Aggregation Propensity of the Human Prion-Like Protein hnRNPA2B1
Kacy R. Paul et al.
MOLECULAR AND CELLULAR BIOLOGY (2017)
Arginine Methylation: The Coming of Age
Romeo S. Blanc et al.
MOLECULAR CELL (2017)
Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics
Steven Boeynaems et al.
MOLECULAR CELL (2017)
Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice
Lindsay A. Becker et al.
NATURE (2017)
Antisense oligonucleotide therapy for spinocerebellar ataxia type 2
Daniel R. Scoles et al.
NATURE (2017)
Neurobiology of axonal transport defects in motor neuron diseases: Opportunities for translational research?
Kurt J. De Vos et al.
NEUROBIOLOGY OF DISEASE (2017)
TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics
Ian R. Mackenzie et al.
NEURON (2017)
Toxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export
Kevin Y. Shi et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2017)
Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation
Tariq Afroz et al.
NATURE COMMUNICATIONS (2017)
ALS Associated Mutations in Matrin 3 Alter Protein-Protein Interactions and Impede mRNA Nuclear Export
Ashley Boehringer et al.
SCIENTIFIC REPORTS (2017)
Phosphorylation of the FUS low-complexity domain disrupts phase separation, aggregation, and toxicity
Zachary Monahan et al.
EMBO JOURNAL (2017)
The C9orf72 repeat size correlates with onset age of disease, DNA methylation and transcriptional downregulation of the promoter
I. Gijselinck et al.
MOLECULAR PSYCHIATRY (2016)
Monomethylated and unmethylated FUS exhibit increased binding to Transportin and distinguish FTLD-FUS from ALS-FUS
Marc Suarez-Calvet et al.
ACTA NEUROPATHOLOGICA (2016)
UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome
Roland Hjerpe et al.
CELL (2016)
ATPase-Modulated Stress Granules Contain a Diverse Proteome and Substructure
Saumya Jain et al.
CELL (2016)
C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles
Kyung-Ha Lee et al.
CELL (2016)
Phosphorylation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Truncated Casein Kinase 1 Triggers Mislocalization and Accumulation of TDP-43
Takashi Nonaka et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2016)
Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins
Antonia Ratti et al.
JOURNAL OF NEUROCHEMISTRY (2016)
Engineering therapeutic protein disaggregases
James Shorter
MOLECULAR BIOLOGY OF THE CELL (2016)
A Surveillance Function of the HSPB8-BAG3-HSP70 Chaperone Complex Ensures Stress Granule Integrity and Dynamism
Massimo Ganassi et al.
MOLECULAR CELL (2016)
Decoding ALS: from genes to mechanism
J. Paul Taylor et al.
NATURE (2016)
NEK1 variants confer susceptibility to amyotrophic lateral sclerosis
Kevin P. Kenna et al.
NATURE GENETICS (2016)
Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs
Jie Jiang et al.
NEURON (2016)
ALS Mutations Disrupt Phase Separation Mediated by α-Helical Structure in the TDP-43 Low-Complexity C-Terminal Domain
Alexander E. Conicella et al.
STRUCTURE (2016)
In Vivo Formation of Vacuolated Multi-phase Compartments Lacking Membranes
Hermann Broder Schmidt et al.
CELL REPORTS (2016)
Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD
Steven Boeynaems et al.
SCIENTIFIC REPORTS (2016)
The LC Domain of hnRNPA2 Adopts Similar Conformations in Hydrogel Polymers, Liquid-like Droplets, and Nuclei
Siheng Xiang et al.
CELL (2015)
Phase Separation by Low Complexity Domains Promotes Stress Granule Assembly and Drives Pathological Fibrillization
Amandine Molliex et al.
CELL (2015)
A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation
Avinash Patel et al.
CELL (2015)
Epidemiology and molecular mechanism of frontotemporal lobar degeneration/amyotrophic lateral sclerosis with repeat expansion mutation in C9orf72
Hiroyuki Ishiura et al.
JOURNAL OF NEUROGENETICS (2015)
Formation and Maturation of Phase-Separated Liquid Droplets by RNA-Binding Proteins
Yuan Lin et al.
MOLECULAR CELL (2015)
Residue-by-Residue View of In Vitro FUS Granules that Bind the C-Terminal Domain of RNA Polymerase II
Kathleen A. Burke et al.
MOLECULAR CELL (2015)
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport
Ke Zhang et al.
NATURE (2015)
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport
Brian D. Freibaum et al.
NATURE (2015)
Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia
Axel Freischmidt et al.
NATURE NEUROSCIENCE (2015)
Exome sequencing in amyotrophic lateral sclerosis identifies risk genes and pathways
Elizabeth T. Cirulli et al.
SCIENCE (2015)
SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis
Francesco Tafuri et al.
FRONTIERS IN CELLULAR NEUROSCIENCE (2015)
An acetylation switch controls TDP-43 function and aggregation propensity
Todd J. Cohen et al.
NATURE COMMUNICATIONS (2015)
Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions
Natalie E. Farrawell et al.
SCIENTIFIC REPORTS (2015)
Stress Granules Modulate SYK to Cause Microglial Cell Dysfunction in Alzheimer's Disease
Soumitra Ghosh et al.
EBIOMEDICINE (2015)
Liquid-Liquid Phase Separation in Biology
Anthony A. Hyman et al.
ANNUAL REVIEW OF CELL AND DEVELOPMENTAL BIOLOGY, VOL 30 (2014)
FUS is Phosphorylated by DNA-PK and Accumulates in the Cytoplasm after DNA Damage
Qiudong Deng et al.
JOURNAL OF NEUROSCIENCE (2014)
Analysis of amyotrophic lateral sclerosis as a multistep process: a population-based modelling study
Ammar AI-Chalabi et al.
LANCET NEUROLOGY (2014)
Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis
Janel O. Johnson et al.
NATURE NEUROSCIENCE (2014)
The role of FUS gene variants in neurodegenerative diseases
Hao Deng et al.
NATURE REVIEWS NEUROLOGY (2014)
Clinicopathologic features of autosomal recessive amyotrophic lateral sclerosis associated with optineurin mutation
Masaki Kamada et al.
NEUROPATHOLOGY (2014)
PASTA 2.0: an improved server for protein aggregation prediction
Ian Walsh et al.
NUCLEIC ACIDS RESEARCH (2014)
Full-length TDP-43 forms toxic amyloid oligomers that are present in frontotemporal lobar dementia-TDP patients
Yu-Sheng Fang et al.
NATURE COMMUNICATIONS (2014)
Novel monoclonal antibodies to normal and pathologically altered human TDP-43 proteins
Linda K. Kwong et al.
ACTA NEUROPATHOLOGICA COMMUNICATIONS (2014)
Protein aggregation in amyotrophic lateral sclerosis
Anna M. Blokhuis et al.
ACTA NEUROPATHOLOGICA (2013)
Inclusions in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), but not FTLD with FUS proteinopathy (FTLD-FUS), have properties of amyloid
Eileen H. Bigio et al.
ACTA NEUROPATHOLOGICA (2013)
Eukaryotic Stress Granules Are Cleared by Autophagy and Cdc48/VCP Function
J. Ross Buchan et al.
CELL (2013)
Phosphorylation-Regulated Binding of RNA Polymerase II to Fibrous Polymers of Low-Complexity Domains
Ilmin Kwon et al.
CELL (2013)
Phase transitions and size scaling of membrane-less organelles
Clifford P. Brangwynne
JOURNAL OF CELL BIOLOGY (2013)
An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study (vol 12, pg 435, 2013)
T. M. Miller et al.
LANCET NEUROLOGY (2013)
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS
Hong Joo Kim et al.
NATURE (2013)
Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons
Wen-Yuan Wang et al.
NATURE NEUROSCIENCE (2013)
Unconventional Translation of C9ORF72 GGGGCC Expansion Generates Insoluble Polypeptides Specific to c9FTD/ALS
Peter E. A. Ash et al.
NEURON (2013)
The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS
Kohji Mori et al.
SCIENCE (2013)
RNA Seeds Higher-Order Assembly of FUS Protein
Jacob C. Schwartz et al.
CELL REPORTS (2013)
An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study
Timothy M. Miller et al.
LANCET NEUROLOGY (2013)
TDP-43 aggregation in neurodegeneration: Are stress granules the key?
Colleen M. Dewey et al.
BRAIN RESEARCH (2012)
Cell-free Formation of RNA Granules: Low Complexity Sequence Domains Form Dynamic Fibers within Hydrogels
Masato Kato et al.
CELL (2012)
Cell-free Formation of RNA Granules: Bound RNAs Identify Features and Components of Cellular Assemblies
Tina W. Han et al.
CELL (2012)
Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS
Dorothee Dormann et al.
EMBO JOURNAL (2012)
Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis
Julien Couthouis et al.
HUMAN MOLECULAR GENETICS (2012)
Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study
Elisa Majounie et al.
LANCET NEUROLOGY (2012)
Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis
Chi-Hong Wu et al.
NATURE (2012)
Valosin-containing protein (VCP) mutations in sporadic amyotrophic lateral sclerosis
Yevgeniya Abramzon et al.
NEUROBIOLOGY OF AGING (2012)
Ataxin-2-Like Is a Regulator of Stress Granules and Processing Bodies
Christian Kaehler et al.
PLOS ONE (2012)
Structural and energetic basis of ALS-causing mutations in the atypical proline-tyrosine nuclear localization signal of the Fused in Sarcoma protein (FUS)
Zi Chao Zhang et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2012)
FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations
Manuela Neumann et al.
BRAIN (2011)
Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients
Teresa Lee et al.
HUMAN MOLECULAR GENETICS (2011)
TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1
Karli K. McDonald et al.
HUMAN MOLECULAR GENETICS (2011)
TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor
Colleen M. Dewey et al.
MOLECULAR AND CELLULAR BIOLOGY (2011)
RNA-binding proteins with prion-like domains in ALS and FTLD-U
Aaron D. Gitler et al.
PRION (2011)
A yeast functional screen predicts new candidate ALS disease genes
Julien Couthouis et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
TDP-43 pathology in sporadic ALS occurs in motor neurons lacking the RNA editing enzyme ADAR2
Hitoshi Aizawa et al.
ACTA NEUROPATHOLOGICA (2010)
ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import
Dorothee Dormann et al.
EMBO JOURNAL (2010)
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
Andrew C. Elden et al.
NATURE (2010)
Mutations of optineurin in amyotrophic lateral sclerosis
Hirofumi Maruyama et al.
NATURE (2010)
TGF-β-mediated phosphorylation of hnRNP E1 induces EMT via transcript-selective translational induction of Dab2 and ILEI
Arindam Chaudhury et al.
NATURE CELL BIOLOGY (2010)
Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue
Liqun Liu-Yesucevitz et al.
PLOS ONE (2010)
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies
Manuela Neumann et al.
ACTA NEUROPATHOLOGICA (2009)
A new subtype of frontotemporal lobar degeneration with FUS pathology
Manuela Neumann et al.
BRAIN (2009)
Variation in aggregation propensities among ALS-associated variants of SOD1: Correlation to human disease
Mercedes Prudencio et al.
HUMAN MOLECULAR GENETICS (2009)
TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity
Brian S. Johnson et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
TDP-43 is recruited to stress granules in conditions of oxidative insult
Claudia Colombrita et al.
JOURNAL OF NEUROCHEMISTRY (2009)
Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity
Yong-Jie Zhang et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
Caroline Vance et al.
SCIENCE (2009)
Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis
T. J. Kwiatkowski et al.
SCIENCE (2009)
Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Lionel M. Igaz et al.
AMERICAN JOURNAL OF PATHOLOGY (2008)
Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Masato Hasegawa et al.
ANNALS OF NEUROLOGY (2008)
The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress response
Mattias K. Andersson et al.
BMC CELL BIOLOGY (2008)
The mammalian Nek1 kinase is involved in primary cilium formation
Ohad Shalom et al.
FEBS LETTERS (2008)
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation
Matthew J. Winton et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
A role for Q/N-rich aggregation-prone regions in P-body localization
Martin A. M. Reijns et al.
JOURNAL OF CELL SCIENCE (2008)
Structural determinants of the cellular localization and shuttling of TDP-43
Youhna M. Ayala et al.
JOURNAL OF CELL SCIENCE (2008)
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
Edor Kabashi et al.
NATURE GENETICS (2008)
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
Jemeen Sreedharan et al.
SCIENCE (2008)
Ataxin-2 interacts with the DEAD/H-box RNA helicase DDX6 and interferes with P-bodies and stress granules
Ute Nonhoff et al.
MOLECULAR BIOLOGY OF THE CELL (2007)
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Tetsuaki Arai et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2006)
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Manuela Neumann et al.
SCIENCE (2006)
TDP-43 binds heterogeneous nuclear ribonucleoprotein A/B through its C-terminal tail - An important region for the inhibition of cystic fibrosis transmembrane conductance regulator exon 9 splicing
E Buratti et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Prediction of sequence-dependent and mutational effects on the aggregation of peptides and proteins
AM Fernandez-Escamilla et al.
NATURE BIOTECHNOLOGY (2004)
Modulation of RNA editing by functional nucleolar sequestration of ADAR2
CL Sansam et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)
Stress granules: sites of mRNA triage that regulate mRNA stability and translatability
N Kedersha et al.
BIOCHEMICAL SOCIETY TRANSACTIONS (2002)
Dynamic shuttling of TIA-1 accompanies the recruitment of mRNA to mammalian stress granules
N Kedersha et al.
JOURNAL OF CELL BIOLOGY (2000)
Point mutation in an AMPA receptor gene rescues lethality in mice deficient in the RNA-editing enzyme ADAR2
M Higuchi et al.
NATURE (2000)