(Q38789192)

6 September 2017

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

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6 September 2017

2

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6 September 2017

Antonia Ratti

1

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PubMed ID

27015757

6 September 2017

language of work or name

English

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publication date

26 March 2016

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Journal of Neurochemistry

6 September 2017

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6 September 2017

138 Suppl 1

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6 September 2017

95-111

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Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations

6 September 2017

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TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

21 January 2018

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Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS.

21 January 2018

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

21 January 2018

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Autoregulation of TDP-43 mRNA levels involves interplay between transcription, splicing, and alternative polyA site selection.

21 January 2018

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

21 January 2018

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Human 75-kDa DNA-pairing protein is identical to the pro-oncoprotein TLS/FUS and is able to promote D-loop formation

21 January 2018

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How do the RNA-binding proteins TDP-43 and FUS relate to amyotrophic lateral sclerosis and frontotemporal degeneration, and to each other?

21 January 2018

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Delocalization of the multifunctional RNA splicing factor TLS/FUS in hippocampal neurones: exclusion from the nucleus and accumulation in dendritic granules and spine heads

21 January 2018

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Stress granules in neurodegeneration--lessons learnt from TAR DNA binding protein of 43 kDa and fused in sarcoma

21 January 2018

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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.

21 January 2018

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Characterization of FUS mutations in amyotrophic lateral sclerosis using RNA-Seq

21 January 2018

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Protein aggregation in amyotrophic lateral sclerosis.

21 January 2018

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

21 January 2018

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TDP-43 overexpression enhances exon 7 inclusion during the survival of motor neuron pre-mRNA splicing

21 January 2018

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Characterization and functional implications of the RNA binding properties of nuclear factor TDP-43, a novel splicing regulator of CFTR exon 9

21 January 2018

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TDP-43: gumming up neurons through protein-protein and protein-RNA interactions

21 January 2018

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Nuclear factor TDP-43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping

21 January 2018

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Nuclear factor TDP-43 can affect selected microRNA levels

21 January 2018

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TDP-43 high throughput screening analyses in neurodegeneration: advantages and pitfalls

21 January 2018

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Evolution of SR protein and hnRNP splicing regulatory factors

21 January 2018

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RNA-binding proteins as molecular links between cancer and neurodegeneration.

21 January 2018

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TDP-43 inclusion bodies formed in bacteria are structurally amorphous, non-amyloid and inherently toxic to neuroblastoma cells

21 January 2018

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

21 January 2018

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Heat-shock protein dysregulation is associated with functional and pathological TDP-43 aggregation

21 January 2018

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Induction of amyloid fibrils by the C-terminal fragments of TDP-43 in amyotrophic lateral sclerosis

21 January 2018

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Neurodegenerative diseases: quantitative predictions of protein-RNA interactions

21 January 2018

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An acetylation switch controls TDP-43 function and aggregation propensity

21 January 2018

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The RNA-binding motif 45 (RBM45) protein accumulates in inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) patients.

21 January 2018

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

21 January 2018

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TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like cells.

21 January 2018

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From transcriptomic to protein level changes in TDP-43 and FUS loss-of-function cell models.

21 January 2018

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

21 January 2018

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TDP-43 regulates β-adducin (Add2) transcript stability.

21 January 2018

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Futsch/MAP1B mRNA is a translational target of TDP-43 and is neuroprotective in a Drosophila model of amyotrophic lateral sclerosis

21 January 2018

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Aggregate formation prevents dTDP-43 neurotoxicity in the Drosophila melanogaster eye.

21 January 2018

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Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma

21 January 2018

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Prion-like disorders: blurring the divide between transmissibility and infectivity

21 January 2018

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Coaggregation of RNA-binding proteins in a model of TDP-43 proteinopathy with selective RGG motif methylation and a role for RRM1 ubiquitination

21 January 2018

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TDP-43 affects splicing profiles and isoform production of genes involved in the apoptotic and mitotic cellular pathways

21 January 2018

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The role of FUS gene variants in neurodegenerative diseases

21 January 2018

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TDP-43 regulates the microprocessor complex activity during in vitro neuronal differentiation

21 January 2018

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Fused in sarcoma (FUS): an oncogene goes awry in neurodegeneration

21 January 2018

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

21 January 2018

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

21 January 2018

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Messenger-RNA-binding proteins and the messages they carry

21 January 2018

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The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth.

21 January 2018

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Transcriptome-wide analysis of TDP-43 binding small RNAs identifies miR-NID1 (miR-8485), a novel miRNA that represses NRXN1 expression

21 January 2018

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Full-length TDP-43 forms toxic amyloid oligomers that are present in frontotemporal lobar dementia-TDP patients.

21 January 2018

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TDP-43 is intercellularly transmitted across axon terminals

21 January 2018

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TDP-43 knockdown impairs neurite outgrowth dependent on its target histone deacetylase 6.

21 January 2018

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TDP-43 regulates global translational yield by splicing of exon junction complex component SKAR.

21 January 2018

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Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.

21 January 2018

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TLS facilitates transport of mRNA encoding an actin-stabilizing protein to dendritic spines

21 January 2018

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The RNA binding protein TLS is translocated to dendritic spines by mGluR5 activation and regulates spine morphology.

21 January 2018

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A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions

21 January 2018

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Identification and characterization of FUS/TLS as a new target of ATM.

21 January 2018

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Mislocalised FUS mutants stall spliceosomal snRNPs in the cytoplasm

21 January 2018

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Evolutionary conservation and expression of human RNA-binding proteins and their role in human genetic disease

21 January 2018

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TDP-43 A315T mutation in familial motor neuron disease

21 January 2018

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TDP-43 regulates Drosophila neuromuscular junctions growth by modulating Futsch/MAP1B levels and synaptic microtubules organization

21 January 2018

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The therapeutic importance of understanding mechanisms of neuronal cell death in neurodegenerative disease

21 January 2018

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Neuronal dark matter: the emerging role of microRNAs in neurodegeneration

21 January 2018

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Long noncoding RNAs(lncRNAs) and the molecular hallmarks of aging

21 January 2018

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The Microprocessor complex mediates the genesis of microRNAs

21 January 2018

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An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity

21 January 2018

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Regulation of MALAT1 expression by TDP43 controls the migration and invasion of non-small cell lung cancer cells in vitro

21 January 2018

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Mechanisms of disease in frontotemporal lobar degeneration: gain of function versus loss of function effects

21 January 2018

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Motor neuron disease and frontotemporal dementia: sometimes related, sometimes not.

21 January 2018

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Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death.

21 January 2018

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TDP-43 associates with stalled ribosomes and contributes to cell survival during cellular stress

21 January 2018

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RNA targets of wild-type and mutant FET family proteins

21 January 2018

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21 January 2018

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

21 January 2018

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TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity

21 January 2018

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Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

21 January 2018

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TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

21 January 2018

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FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis

21 January 2018

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Detection of TDP-43 oligomers in frontotemporal lobar degeneration-TDP.

21 January 2018

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

21 January 2018

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Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA.

21 January 2018

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

21 January 2018

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The RNA-binding protein TDP-43 selectively disrupts microRNA-1/206 incorporation into the RNA-induced silencing complex.

21 January 2018

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

21 January 2018

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

21 January 2018

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Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis

21 January 2018

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

21 January 2018

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A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.

21 January 2018

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TARDBP and FUS mutations associated with amyotrophic lateral sclerosis: summary and update

21 January 2018

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Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key?

21 January 2018

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Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic

21 January 2018

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Identification of a subnuclear body involved in sequence-specific cytokine RNA processing.

21 January 2018

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Soluble forms of polyQ-expanded huntingtin rather than large aggregates cause endoplasmic reticulum stress

21 January 2018

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Transposable elements in TDP-43-mediated neurodegenerative disorders

21 January 2018

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Stress granules as crucibles of ALS pathogenesis.

21 January 2018

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A liver-enriched long non-coding RNA, lncLSTR, regulates systemic lipid metabolism in mice.

21 January 2018

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RBM45 homo-oligomerization mediates association with ALS-linked proteins and stress granules

21 January 2018

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ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.

21 January 2018

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Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis

21 January 2018

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Long non-coding RNA gadd7 interacts with TDP-43 and regulates Cdk6 mRNA decay

21 January 2018

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Long noncoding RNAs in TDP-43 and FUS/TLS-related frontotemporal lobar degeneration (FTLD).

21 January 2018

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Molecular basis of UG-rich RNA recognition by the human splicing factor TDP-43

21 January 2018

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FET proteins in frontotemporal dementia and amyotrophic lateral sclerosis

21 January 2018

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Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations.

21 January 2018

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TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

21 January 2018

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MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis.

21 January 2018

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TDP-43 regulates the mammalian spinogenesis through translational repression of Rac1.

21 January 2018

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The RNA-binding protein fused in sarcoma (FUS) functions downstream of poly(ADP-ribose) polymerase (PARP) in response to DNA damage

21 January 2018

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Position-specific binding of FUS to nascent RNA regulates mRNA length

21 January 2018

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TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1.

21 January 2018

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TDP-43 functions within a network of hnRNP proteins to inhibit the production of a truncated human SORT1 receptor

21 January 2018

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"Structural characterization of the minimal segment of TDP-43 competent for aggregation".

21 January 2018

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Structural Evidence of Amyloid Fibril Formation in the Putative Aggregation Domain of TDP-43.

21 January 2018

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hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations

21 January 2018

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FUS stimulates microRNA biogenesis by facilitating co-transcriptional Drosha recruitment

21 January 2018

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Genomic structure of the human RBP56/hTAFII68 and FUS/TLS genes

21 January 2018

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Shared Molecular Mechanisms in Alzheimer's Disease and Amyotrophic Lateral Sclerosis: Neurofilament-Dependent Transport of sAPP, FUS, TDP-43 and SOD1, with Endoplasmic Reticulum-Like Tubules

21 January 2018

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

21 January 2018

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Identification of RNA bound to the TDP-43 ribonucleoprotein complex in the adult mouse brain.

21 January 2018

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Frontotemporal lobar degeneration and amyotrophic lateral sclerosis: molecular similarities and differences

21 January 2018

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

21 January 2018

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A new subtype of frontotemporal lobar degeneration with FUS pathology

21 January 2018

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FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations

21 January 2018

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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

21 January 2018

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Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration

21 January 2018

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Prion-like properties of pathological TDP-43 aggregates from diseased brains

21 January 2018

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RNA-binding proteins in neurodegeneration: Seq and you shall receive

21 January 2018

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FUS-mediated alternative splicing in the nervous system: consequences for ALS and FTLD.

21 January 2018

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Tat-activating regulatory DNA-binding protein regulates glycolysis in hepatocellular carcinoma by regulating the platelet isoform of phosphofructokinase through microRNA 520.

21 January 2018

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Prion-like spread of protein aggregates in neurodegeneration

21 January 2018

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

21 January 2018

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Deciphering spreading mechanisms in amyotrophic lateral sclerosis: clinical evidence and potential molecular processes

21 January 2018

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Misregulation of human sortilin splicing leads to the generation of a nonfunctional progranulin receptor

21 January 2018

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Neurodegeneration the RNA way

21 January 2018

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The role of long non-coding RNAs in neurodevelopment, brain function and neurological disease

21 January 2018

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TDP-43 skeins show properties of amyloid in a subset of ALS cases

21 January 2018

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

21 January 2018

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TBPH/TDP-43 modulates translation of Drosophila futsch mRNA through an UG-rich sequence within its 5'UTR.

21 January 2018

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PARP-1 dependent recruitment of the amyotrophic lateral sclerosis-associated protein FUS/TLS to sites of oxidative DNA damage.

21 January 2018

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Delineation of the core aggregation sequences of TDP-43 C-terminal fragment.

21 January 2018

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Self-assembling properties of peptides derived from TDP-43 C-terminal fragment

21 January 2018

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Protein arginine methyltransferase 1 and 8 interact with FUS to modify its sub-cellular distribution and toxicity in vitro and in vivo

21 January 2018

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FUS binds the CTD of RNA polymerase II and regulates its phosphorylation at Ser2.

21 January 2018

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FUS is sequestered in nuclear aggregates in ALS patient fibroblasts

21 January 2018

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The function of RNA-binding proteins at the synapse: implications for neurodegeneration

21 January 2018

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TDP-43 is a developmentally regulated protein essential for early embryonic development

21 January 2018

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Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexes

21 January 2018

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Multiplex SILAC analysis of a cellular TDP-43 proteinopathy model reveals protein inclusions associated with SUMOylation and diverse polyubiquitin chains

21 January 2018

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Modelling FUSopathies: focus on protein aggregation

21 January 2018

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Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice

21 January 2018

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Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasm

21 January 2018

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Compromised paraspeckle formation as a pathogenic factor in FUSopathies

21 January 2018

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Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALS

21 January 2018

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Aberrant assembly of RNA recognition motif 1 links to pathogenic conversion of TAR DNA-binding protein of 43 kDa (TDP-43)

21 January 2018

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Review: Prion-like mechanisms of transactive response DNA binding protein of 43 kDa (TDP-43) in amyotrophic lateral sclerosis (ALS).

21 January 2018

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TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

21 January 2018

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Proteomic analyses reveal that loss of TDP-43 affects RNA processing and intracellular transport

21 January 2018

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TDP-43, an ALS linked protein, regulates fat deposition and glucose homeostasis

21 January 2018

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TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein

21 January 2018

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Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS

21 January 2018

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The influence of pathological mutations and proline substitutions in TDP-43 glycine-rich peptides on its amyloid properties and cellular toxicity.

21 January 2018

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

21 January 2018

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Making and breaking synapses through local mRNA regulation

21 January 2018

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TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation.

21 January 2018

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

21 January 2018

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Analysis of alternative splicing associated with aging and neurodegeneration in the human brain.

21 January 2018

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Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations

21 January 2018

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

21 January 2018

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FUS regulates AMPA receptor function and FTLD/ALS-associated behaviour via GluA1 mRNA stabilization

21 January 2018

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Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6

21 January 2018

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TDP-43-mediated neurodegeneration: towards a loss-of-function hypothesis?

21 January 2018

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Role of stress granules and RNA-binding proteins in neurodegeneration: a mini-review

21 January 2018

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MicroRNAs: newcomers into the ALS picture

21 January 2018

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TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor

21 January 2018

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Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription

21 January 2018

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The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span.

21 January 2018

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Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons.

21 January 2018

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Nucleic acid-binding specificity of human FUS protein

21 January 2018

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TDP-43-based animal models of neurodegeneration: new insights into ALS pathology and pathophysiology

21 January 2018

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Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA.

21 January 2018

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RNA targets of TDP-43 identified by UV-CLIP are deregulated in ALS.

21 January 2018

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Does a loss of TDP-43 function cause neurodegeneration?

21 January 2018

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The effect of PRMT1-mediated arginine methylation on the subcellular localization, stress granules, and detergent-insoluble aggregates of FUS/TLS.

21 January 2018

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FUS-SMN protein interactions link the motor neuron diseases ALS and SMA

21 January 2018

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Self-assembled FUS binds active chromatin and regulates gene transcription.

21 January 2018

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The RNA-binding protein Fus directs translation of localized mRNAs in APC-RNP granules

21 January 2018

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Myosin-Va facilitates the accumulation of mRNA/protein complex in dendritic spines.

21 January 2018

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FUS functions in coupling transcription to splicing by mediating an interaction between RNAP II and U1 snRNP

21 January 2018

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U1 snRNP is mislocalized in ALS patient fibroblasts bearing NLS mutations in FUS and is required for motor neuron outgrowth in zebrafish

21 January 2018

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SOD1 protein aggregates stimulate macropinocytosis in neurons to facilitate their propagation

21 January 2018

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Downregulation of microRNA-9 in iPSC-derived neurons of FTD/ALS patients with TDP-43 mutations.

21 January 2018

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ALS-associated FUS mutations result in compromised FUS alternative splicing and autoregulation

21 January 2018

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21 January 2018

Identifiers

DOI

10.1111/JNC.13625

1 reference

6 September 2017

1 reference

PubMed ID

27015757