RNA-mediated toxicity in neurodegenerative disease.

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Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS. Acta Neuropathol (2013) 1.95

Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood. Acta Neuropathol (2013) 1.52

Mechanisms of toxicity in C9FTLD/ALS. Acta Neuropathol (2014) 1.35

Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders. Hum Mol Genet (2013) 1.21

ALS as a distal axonopathy: molecular mechanisms affecting neuromuscular junction stability in the presymptomatic stages of the disease. Front Neurosci (2014) 1.10

Characterization of DNA hypermethylation in the cerebellum of c9FTD/ALS patients. Brain Res (2014) 0.93

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Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLD. Hum Mol Genet (2013) 0.89

Combating neurodegenerative disease with chemical probes and model systems. Nat Chem Biol (2014) 0.85

RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosis. Front Cell Neurosci (2014) 0.82

ALS and FTD: an epigenetic perspective. Acta Neuropathol (2016) 0.81

GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function. Elife (2015) 0.81

Protein-RNA Networks Regulated by Normal and ALS-Associated Mutant HNRNPA2B1 in the Nervous System. Neuron (2016) 0.79

Endocrine disrupters: the new players able to affect the epigenome. Front Cell Dev Biol (2015) 0.79

Aberrant RNA homeostasis in amyotrophic lateral sclerosis: potential for new therapeutic targets? Neurodegener Dis Manag (2014) 0.78

A network of RNA and protein interactions in Fronto Temporal Dementia. Front Mol Neurosci (2015) 0.77

Emerging Infections of CNS: Avian Influenza A Virus, Rift Valley Fever Virus and Human Parechovirus. Brain Pathol (2015) 0.76

Repeat Associated Non-AUG Translation (RAN Translation) Dependent on Sequence Downstream of the ATXN2 CAG Repeat. PLoS One (2015) 0.76

Pur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity. Acta Neuropathol (2016) 0.75

Proteostasis and Diseases of the Motor Unit. Front Mol Neurosci (2016) 0.75

A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects. Dis Model Mech (2016) 0.75

Neurotrophic effects of progranulin in vivo in reversing motor neuron defects caused by over or under expression of TDP-43 or FUS. PLoS One (2017) 0.75

Drosophila melanogaster As a Model Organism to Study RNA Toxicity of Repeat Expansion-Associated Neurodegenerative and Neuromuscular Diseases. Front Cell Neurosci (2017) 0.75

Decoding Parkinson's Disease Pathogenesis: The Role of Deregulated mRNA Translation. J Parkinsons Dis (2016) 0.75

Expression of microRNAs in human post-mortem amyotrophic lateral sclerosis spinal cords provides insight into disease mechanisms. Mol Cell Neurosci (2015) 0.75

M1 RNA is important for the in-cell solubility of its cognate C5 protein: Implications for RNA-mediated protein folding. RNA Biol (2015) 0.75

Conserved DNA methylation combined with differential frontal cortex and cerebellar expression distinguishes C9orf72-associated and sporadic ALS, and implicates SERPINA1 in disease. Acta Neuropathol (2017) 0.75

Genetic mutations in RNA-binding proteins and their roles in ALS. Hum Genet (2017) 0.75

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science (2006) 27.96

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron (2011) 20.15

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron (2011) 18.73

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TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science (2008) 14.09

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science (2009) 13.45

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science (2009) 12.99

CTCF mediates methylation-sensitive enhancer-blocking activity at the H19/Igf2 locus. Nature (2000) 12.97

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Widespread occurrence of antisense transcription in the human genome. Nat Biotechnol (2003) 8.54

An unstable triplet repeat in a gene related to myotonic muscular dystrophy. Science (1992) 8.20

Epigenetic silencing of tumour suppressor gene p15 by its antisense RNA. Nature (2008) 7.64

Myotonic dystrophy type 2 caused by a CCTG expansion in intron 1 of ZNF9. Science (2001) 7.34

Disruption of splicing regulated by a CUG-binding protein in myotonic dystrophy. Science (1998) 6.67

Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy. EMBO J (2000) 6.56

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CTCF mediates long-range chromatin looping and local histone modification in the beta-globin locus. Genes Dev (2006) 6.32

The antisense transcriptomes of human cells. Science (2008) 6.29

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Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. Nat Neurosci (2011) 6.01

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A muscleblind knockout model for myotonic dystrophy. Science (2003) 5.93

Myotonic dystrophy in transgenic mice expressing an expanded CUG repeat. Science (2000) 5.85

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CTCF tethers an insulator to subnuclear sites, suggesting shared insulator mechanisms across species. Mol Cell (2004) 5.51

Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2-21.3. Brain (2006) 5.36

TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis. Lancet Neurol (2008) 5.33

A locus on chromosome 9p confers susceptibility to ALS and frontotemporal dementia. Neurology (2006) 5.29

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Characterizing the RNA targets and position-dependent splicing regulation by TDP-43. Nat Neurosci (2011) 5.24

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. Lancet Neurol (2011) 5.01

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SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing. Cell (2008) 4.54

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Antisense transcription and heterochromatin at the DM1 CTG repeats are constrained by CTCF. Mol Cell (2005) 4.07

Foci of trinucleotide repeat transcripts in nuclei of myotonic dystrophy cells and tissues. J Cell Biol (1995) 4.04

Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease. Front Biosci (2008) 4.01

Bidirectional expression of CUG and CAG expansion transcripts and intranuclear polyglutamine inclusions in spinocerebellar ataxia type 8. Nat Genet (2006) 3.87

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. Acta Neuropathol (2011) 3.73

Clinical, neuroimaging and neuropathological features of a new chromosome 9p-linked FTD-ALS family. J Neurol Neurosurg Psychiatry (2010) 3.66

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity. Proc Natl Acad Sci U S A (2009) 3.65

Short GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophy. Nat Genet (1998) 3.62

Non-ATG-initiated translation directed by microsatellite expansions. Proc Natl Acad Sci U S A (2010) 3.58

Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation. J Biol Chem (2008) 3.56

Glycyl tRNA synthetase mutations in Charcot-Marie-Tooth disease type 2D and distal spinal muscular atrophy type V. Am J Hum Genet (2003) 3.55

Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43. J Neurosci (2007) 3.52

Structural diversity and functional implications of the eukaryotic TDP gene family. Genomics (2004) 3.51

A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity. Proc Natl Acad Sci U S A (2008) 3.46

Over 20% of human transcripts might form sense-antisense pairs. Nucleic Acids Res (2004) 3.45

Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain (2012) 3.43

An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMN. Hum Mol Genet (2000) 3.41

TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci U S A (2010) 3.39

Three proteins, MBNL, MBLL and MBXL, co-localize in vivo with nuclear foci of expanded-repeat transcripts in DM1 and DM2 cells. Hum Mol Genet (2002) 3.34

Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72. Acta Neuropathol (2011) 3.34

TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity. J Biol Chem (2009) 3.29

Muscleblind localizes to nuclear foci of aberrant RNA in myotonic dystrophy types 1 and 2. Hum Mol Genet (2001) 3.27

Myotonic dystrophy type 1 is associated with nuclear foci of mutant RNA, sequestration of muscleblind proteins and deregulated alternative splicing in neurons. Hum Mol Genet (2004) 3.21

Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Ann Neurol (2008) 3.13

Disrupted function and axonal distribution of mutant tyrosyl-tRNA synthetase in dominant intermediate Charcot-Marie-Tooth neuropathy. Nat Genet (2006) 3.04

hnRNP K: one protein multiple processes. Bioessays (2004) 2.96

Protein composition of the intranuclear inclusions of FXTAS. Brain (2005) 2.95

CTCF-binding sites flank CTG/CAG repeats and form a methylation-sensitive insulator at the DM1 locus. Nat Genet (2001) 2.93

TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor. J Neurochem (2007) 2.91

TDP-43 regulates its mRNA levels through a negative feedback loop. EMBO J (2010) 2.90

FMR1 RNA within the intranuclear inclusions of fragile X-associated tremor/ataxia syndrome (FXTAS). RNA Biol (2004) 2.90

Three families with amyotrophic lateral sclerosis and frontotemporal dementia with evidence of linkage to chromosome 9p. Arch Neurol (2007) 2.89

Structural determinants of the cellular localization and shuttling of TDP-43. J Cell Sci (2008) 2.79

Decreased expression of myotonin-protein kinase messenger RNA and protein in adult form of myotonic dystrophy. Science (1993) 2.75

Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis. J Neurosci (2010) 2.73

Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs. PLoS One (2007) 2.70

RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1. J Biol Chem (2000) 2.65

Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. J Neurosci (2010) 2.60

Large expansion of the ATTCT pentanucleotide repeat in spinocerebellar ataxia type 10. Nat Genet (2000) 2.60

Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy. Hum Mol Genet (2005) 2.59

Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice. J Clin Invest (2011) 2.58

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Mice lacking the myotonic dystrophy protein kinase develop a late onset progressive myopathy. Nat Genet (1996) 2.46

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RNA gain-of-function in spinocerebellar ataxia type 8. PLoS Genet (2009) 2.45

Overexpression of CUG triplet repeat-binding protein, CUGBP1, in mice inhibits myogenesis. J Biol Chem (2004) 2.43

Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patients. EMBO J (2010) 2.43

Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron (2013) 5.91

Inhibitors of leucine-rich repeat kinase-2 protect against models of Parkinson's disease. Nat Med (2010) 4.45

Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis. PLoS Genet (2008) 3.66

Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity. Proc Natl Acad Sci U S A (2009) 3.65

Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43. J Neurosci (2007) 3.52

The high-affinity HSP90-CHIP complex recognizes and selectively degrades phosphorylated tau client proteins. J Clin Invest (2007) 3.35

RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention. Neuron (2013) 3.25

Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion. Sci Transl Med (2013) 2.89

Structural determinants of the cellular localization and shuttling of TDP-43. J Cell Sci (2008) 2.79

Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. J Neurosci (2010) 2.60

Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue. PLoS One (2010) 2.49

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Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS. Acta Neuropathol (2013) 1.95

TMEM106B protects C9ORF72 expansion carriers against frontotemporal dementia. Acta Neuropathol (2014) 1.90

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C9ORF72 repeat expansions in cases with previously identified pathogenic mutations. Neurology (2013) 1.70

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Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood. Acta Neuropathol (2013) 1.52

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