Related references
Note: Only part of the references are listed.Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA
Andreas C. Woerner et al.
SCIENCE (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)
C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins
Yong-Jie Zhang et al.
NATURE NEUROSCIENCE (2016)
Prevalence of brain and spinal cord inclusions, including dipeptide repeat proteins, in patients with the C9ORF72 hexanucleotide repeat expansion: a systematic neuropathological review
L. J. Schipper et al.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2016)
Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD
Steven Boeynaems et al.
SCIENTIFIC REPORTS (2016)
Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43
Adam K. Walker et al.
ACTA NEUROPATHOLOGICA (2015)
Isoform-Specific Antibodies Reveal Distinct Subcellular Localizations of C9orf72 in Amyotrophic Lateral Sclerosis
Shangxi Xiao et al.
ANNALS OF NEUROLOGY (2015)
Nuclear localization signals for four distinct karyopherin-β nuclear import systems
Michael Soniat et al.
BIOCHEMICAL JOURNAL (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)
Phosphorylation of C-terminal tyrosine residue 526 in FUS impairs its nuclear import
Simona Darovic et al.
JOURNAL OF CELL SCIENCE (2015)
Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS
Simona Rossi et al.
JOURNAL OF CELL SCIENCE (2015)
Cytoplasmic hGle1A regulates stress granules by modulation of translation
Aditi et al.
MOLECULAR BIOLOGY OF THE CELL (2015)
Formation and Maturation of Phase-Separated Liquid Droplets by RNA-Binding Proteins
Yuan Lin 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)
Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS
Ana Jovicic et al.
NATURE NEUROSCIENCE (2015)
Control of mammalian gene expression by selective mRNA export
Vihandha O. Wickramasinghe et al.
NATURE REVIEWS MOLECULAR CELL BIOLOGY (2015)
ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function
Tetsuro Murakami et al.
NEURON (2015)
PROTEOMIC ANALYSES REVEAL THAT LOSS OF TDP-43 AFFECTS RNA PROCESSING AND INTRACELLULAR TRANSPORT
M. Stalekar et al.
NEUROSCIENCE (2015)
Components and regulation of nuclear transport processes
Bastien Cautain et al.
FEBS JOURNAL (2015)
Deleterious mutations in the essential mRNA metabolism factor, hGle1, in amyotrophic lateral sclerosis
Hannah M. Kaneb et al.
HUMAN MOLECULAR GENETICS (2015)
The widening spectrum of C9ORF72-related disease; genotype/phenotype correlations and potential modifiers of clinical phenotype
Johnathan Cooper-Knock et al.
ACTA NEUROPATHOLOGICA (2014)
TDP-43 is a key player in the clinical features associated with Alzheimer's disease
Keith A. Josephs et al.
ACTA NEUROPATHOLOGICA (2014)
Transportin-1 and Transportin-2: Protein nuclear import and beyond
Laure Twyffels et al.
FEBS LETTERS (2014)
Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones
Maria Udan-Johns et al.
HUMAN MOLECULAR GENETICS (2014)
C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking
Manal A. Farg et al.
HUMAN MOLECULAR GENETICS (2014)
Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD
Michael E. Ward et al.
JOURNAL OF EXPERIMENTAL MEDICINE (2014)
Reduced C9orf72 protein levels in frontal cortex of amyotrophic lateral sclerosis and frontotemporal degeneration brain with the C9ORF72 hexanucleotide repeat expansion
Adrian J. Waite et al.
NEUROBIOLOGY OF AGING (2014)
Unconventional features of C9ORF72 expanded repeat in amyotrophic lateral sclerosis and frontotemporal lobar degeneration
Sabina Vatovec et al.
NEUROBIOLOGY OF AGING (2014)
C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci
Sarah Mizielinska et al.
ACTA NEUROPATHOLOGICA (2013)
Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins
Kohji Mori et al.
ACTA NEUROPATHOLOGICA (2013)
Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS
Tania F. Gendron et al.
ACTA NEUROPATHOLOGICA (2013)
The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs
Timothy P. Levine et al.
BIOINFORMATICS (2013)
Chtop is a component of the dynamic TREX mRNA export complex
Chung-Te Chang et al.
EMBO JOURNAL (2013)
ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules
Caroline Vance et al.
HUMAN MOLECULAR GENETICS (2013)
Mutations in protein N-arginine methyltransferases are not the cause of FTLD-FUS
Thomas A. Ravenscroft et al.
NEUROBIOLOGY OF AGING (2013)
RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention
Christopher J. Donnelly et al.
NEURON (2013)
Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis
Shuo-Chien Ling et al.
NEURON (2013)
Transportin 1 colocalization with Fused in Sarcoma (FUS) inclusions is not characteristic for amyotrophic lateral sclerosis-FUS confirming disrupted nuclear import of mutant FUS and distinguishing it from frontotemporal lobar degeneration with FUS inclusions
C. Troakes et al.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2013)
Nuclear carrier and RNA-binding proteins in frontotemporal lobar degeneration associated with fused in sarcoma (FUS) pathological changes
Y. S. Davidson et al.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2013)
Protein Arginine Methyltransferase 1 and 8 Interact with FUS to Modify Its Sub-Cellular Distribution and Toxicity In Vitro and In Vivo
Chiara Scaramuzzino et al.
PLOS ONE (2013)
Identification of Novel Stress Granule Components That Are Involved in Nuclear Transport
Hicham Mahboubi et al.
PLOS ONE (2013)
RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia
Tao Zu et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)
The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS
Kohji Mori et al.
SCIENCE (2013)
Transportin 1 accumulates specifically with FET proteins but no other transportin cargos in FTLD-FUS and is absent in FUS inclusions in ALS with FUS mutations
Manuela Neumann et al.
ACTA NEUROPATHOLOGICA (2012)
The genetics and neuropathology of frontotemporal lobar degeneration
Anne Sieben et al.
ACTA NEUROPATHOLOGICA (2012)
Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS
Dorothee Dormann et al.
EMBO JOURNAL (2012)
Domains involved in TAF15 subcellular localisation: Dependence on cell type and ongoing transcription
Marija Marko et al.
GENE (2012)
Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations
Miranda L. Tradewell et al.
HUMAN MOLECULAR GENETICS (2012)
A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study
Ilse Gijselinck et al.
LANCET NEUROLOGY (2012)
Mutations in the RNA exosome component gene EXOSC3 cause pontocerebellar hypoplasia and spinal motor neuron degeneration
Jijun Wan et al.
NATURE GENETICS (2012)
An MND/ALS phenotype associated with C9orf72 repeat expansion: Abundant p62-positive, TDP-43-negative inclusions in cerebral cortex, hippocampus and cerebellum but without associated cognitive decline
Claire Troakes et al.
NEUROPATHOLOGY (2012)
The Effect of PRMT1-Mediated Arginine Methylation on the Subcellular Localization, Stress Granules, and Detergent-Insoluble Aggregates of FUS/TLS
Atsushi Yamaguchi et al.
PLOS ONE (2012)
FUS-NLS/Transportin 1 Complex Structure Provides Insights into the Nuclear Targeting Mechanism of FUS and the Implications in ALS
Chunyan Niu et al.
PLOS ONE (2012)
TREX exposes the RNA-binding domain of Nxf1 to enable mRNA export
Nicolas Viphakone et al.
NATURE COMMUNICATIONS (2012)
Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain
Boris Rogelj et al.
SCIENTIFIC REPORTS (2012)
Discovery of Novel DENN Proteins: Implications for the Evolution of Eukaryotic Intracellular Membrane Structures and Human Disease
Dapeng Zhang et al.
Frontiers in Genetics (2012)
Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutation
Ian R. A. Mackenzie et al.
ACTA NEUROPATHOLOGICA (2011)
p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS
Safa Al-Sarraj et al.
ACTA NEUROPATHOLOGICA (2011)
The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene
Julie S. Snowden et al.
ACTA NEUROPATHOLOGICA (2011)
Transportin1: a marker of FTLD-FUS
Jack Brelstaff et al.
ACTA NEUROPATHOLOGICA (2011)
Nuclear import by karyopherin-βs: Recognition and inhibition
Yuh Min Chook et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2011)
Molecular basis for specificity of nuclear import and prediction of nuclear localization
Mary Marfori et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2011)
A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies
Tammaryn Lashley et al.
BRAIN (2011)
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)
TDP-43 regulates its mRNA levels through a negative feedback loop
Youhna M. Ayala et al.
EMBO JOURNAL (2011)
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43
Magdalini Polymenidou et al.
NATURE NEUROSCIENCE (2011)
Characterizing the RNA targets and position-dependent splicing regulation by TDP-43
James R. Tollervey et al.
NATURE NEUROSCIENCE (2011)
Nuclear localization sequence of FUS and induction of stress granules by ALS mutants
Jozsef Gal et al.
NEUROBIOLOGY OF AGING (2011)
Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
Mariely DeJesus-Hernandez et al.
NEURON (2011)
A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
Alan E. Renton et al.
NEURON (2011)
FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration
Hazel Urwin et al.
ACTA NEUROPATHOLOGICA (2010)
Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration
Agnes L. Nishimura et al.
BRAIN (2010)
ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import
Dorothee Dormann et al.
EMBO JOURNAL (2010)
Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules
Daryl A. Bosco et al.
HUMAN MOLECULAR GENETICS (2010)
FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis
Ian P. Blair et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2010)
FUS GENE MUTATIONS IN FAMILIAL AND SPORADIC AMYOTROPHIC LATERAL SCLEROSIS
Rosa Rademakers et al.
MUSCLE & NERVE (2010)
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease
Manuela Neumann et al.
ACTA NEUROPATHOLOGICA (2009)
A new subtype of frontotemporal lobar degeneration with FUS pathology
Manuela Neumann et al.
BRAIN (2009)
Age-Dependent Deterioration of Nuclear Pore Complexes Causes a Loss of Nuclear Integrity in Postmitotic Cells
Maximiliano A. D'Angelo et al.
CELL (2009)
PRMT1 mediated methylation of TAF15 is required for its positive gene regulatory function
Laure Jobert et al.
EXPERIMENTAL CELL RESEARCH (2009)
Nuclear Contour Irregularity and Abnormal Transporter Protein Distribution in Anterior Horn Cells in Amyotrophic Lateral Sclerosis
Yoshimi Kinoshita et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2009)
Proteomic Analysis of Methylarginine-Containing Proteins in HeLa Cells by Two-Dimensional Gel Electrophoresis and Immunoblotting with a Methylarginine-Specific Antibody
Chien-Jen Hung et al.
PROTEIN JOURNAL (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)
Binding Site Distribution of Nuclear Transport Receptors and Transport Complexes in Single Nuclear Pore Complexes
Martin Kahms et al.
TRAFFIC (2009)
Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease
Emanuele Buratti et al.
FRONTIERS IN BIOSCIENCE-LANDMARK (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)
Structural determinants of the cellular localization and shuttling of TDP-43
Youhna M. Ayala et al.
JOURNAL OF CELL SCIENCE (2008)
Mutations in mRNA export mediator GLE1 result in a fetal motoneuron disease
Heidi O. Nousiainen et al.
NATURE GENETICS (2008)
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
Jemeen Sreedharan et al.
SCIENCE (2008)
Crossing the nuclear envelope: Hierarchical regulation of nucleocytoplasmic transport
Laura J. Terry et al.
SCIENCE (2007)
Exporting RNA from the nucleus to the cytoplasm
Alwin Koehler et al.
NATURE REVIEWS MOLECULAR CELL BIOLOGY (2007)
TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease
Linda K. Kwong et al.
ACTA NEUROPATHOLOGICA (2007)
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations
Manuela Neumann et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2007)
Identification and characterization of the nuclear localization/retention signal in the EWS proto-oncoprotein
Rouzanna P. Zakaryan et al.
JOURNAL OF MOLECULAR BIOLOGY (2006)
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Manuela Neumann et al.
SCIENCE (2006)
Rules for nuclear localization sequence recognition by karyopherinβ2
Brittany J. Lee et al.
CELL (2006)
Activation of the DExD/H-box protein Dbp5 by the nuclear-pore protein Gle1 and its coactivator InsP6 is required for mRNA export
Christine S. Weirich et al.
NATURE CELL BIOLOGY (2006)
Inositol hexakisphosphate and Gle1 activate the DEAD-box protein Dbp5 for nuclear mRNA export
Abel R. Alcazar-Roman et al.
NATURE CELL BIOLOGY (2006)
Transcriptional down-regulation through nuclear exclusion of EWS methylated by PRMT1
N Araya et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2005)
Mechanisms of receptor-mediated nuclear import and nuclear export
LF Pemberton et al.
TRAFFIC (2005)
Unstable mutants in the peripheral endosomal membrane component ALS2 cause early-onset motor neuron disease
K Yamanaka et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)
Detection of arginine dimethylated peptides by parallel precursor ion scanning mass spectrometry in positive ion mode
J Rappsilber et al.
ANALYTICAL CHEMISTRY (2003)
Proteomic analysis of the mammalian nuclear pore complex
JA Cronshaw et al.
JOURNAL OF CELL BIOLOGY (2002)
The protein Aly links pre-messenger-RNA splicing to nuclear export in metazoans
ZL Zhou et al.
NATURE (2000)
Share Paper
