Published in Neuron on December 17, 2014
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GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport. Nature (2015) 1.77
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A C9ORF72/SMCR8-containing complex regulates ULK1 and plays a dual role in autophagy. Sci Adv (2016) 0.83
Linking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis. Neurotherapeutics (2015) 0.83
Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation. Hum Mol Genet (2016) 0.83
Drosophila as an In Vivo Model for Human Neurodegenerative Disease. Genetics (2015) 0.83
The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains. Elife (2016) 0.82
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Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition. EMBO Rep (2016) 0.80
ALS mutant SOD1 interacts with G3BP1 and affects stress granule dynamics. Acta Neuropathol (2016) 0.80
Probing disorders of the nervous system using reprogramming approaches. EMBO J (2015) 0.79
C9ORF72 Regulates Stress Granule Formation and Its Deficiency Impairs Stress Granule Assembly, Hypersensitizing Cells to Stress. Mol Neurobiol (2016) 0.79
C9ORF72 interaction with cofilin modulates actin dynamics in motor neurons. Nat Neurosci (2016) 0.79
Fluid-Based Biomarkers for Amyotrophic Lateral Sclerosis. Neurotherapeutics (2016) 0.78
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Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins. Neuropathol Appl Neurobiol (2015) 0.78
Glycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegeneration. Mol Neurodegener (2017) 0.77
Inside out: the role of nucleocytoplasmic transport in ALS and FTLD. Acta Neuropathol (2016) 0.77
Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics. Mol Cell (2017) 0.77
Cell-based screening: extracting meaning from complex data. Neuron (2015) 0.77
Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity. PLoS One (2016) 0.76
Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene. Neurosci Lett (2016) 0.76
A PR plug for the nuclear pore in amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2017) 0.75
Control of mRNA Translation in ALS Proteinopathy. Front Mol Neurosci (2017) 0.75
Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD. Hum Mol Genet (2016) 0.75
Forward Genetic Screen in Caenorhabditis elegans Suggests F57A10.2 and acp-4 As Suppressors of C9ORF72 Related Phenotypes. Front Mol Neurosci (2016) 0.75
Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration. Acta Neuropathol Commun (2017) 0.75
C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten. Hum Mol Genet (2017) 0.75
The Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded C9ORF72 Inhibits the Proteasome. eNeuro (2017) 0.75
New approaches for direct conversion of patient fibroblasts into neural cells. Brain Res (2015) 0.75
Stress granules at the intersection of autophagy and ALS. Brain Res (2016) 0.75
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Repeat-associated non-AUG translation from antisense CCG repeats in fragile X tremor/ataxia syndrome. Ann Neurol (2016) 0.75
A low absolute number of expanded transcripts is involved in myotonic dystrophy type 1 manifestation in muscle. Hum Mol Genet (2016) 0.75
Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder. EMBO J (2017) 0.75
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secHsp70 as a tool to approach amyloid-β42 and other extracellular amyloids. Fly (Austin) (2017) 0.75
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Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Neuron (2013) 5.91
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Conversion of mouse and human fibroblasts into functional spinal motor neurons. Cell Stem Cell (2011) 4.65
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C9orf72 nucleotide repeat structures initiate molecular cascades of disease. Nature (2014) 3.59
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Non-cell-autonomous effect of human SOD1 G37R astrocytes on motor neurons derived from human embryonic stem cells. Cell Stem Cell (2008) 3.34
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention. Neuron (2013) 3.25
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Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study. Lancet Neurol (2012) 3.04
C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins. Science (2014) 2.78
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Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells. Science (2014) 2.43
RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia. Proc Natl Acad Sci U S A (2013) 2.31
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Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration. Proc Natl Acad Sci U S A (2013) 2.30
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Intracellular and trans-synaptic regulation of glutamatergic synaptogenesis by EphB receptors. J Neurosci (2006) 2.17
The disease-associated r(GGGGCC)n repeat from the C9orf72 gene forms tract length-dependent uni- and multimolecular RNA G-quadruplex structures. J Biol Chem (2013) 1.97
Hypermethylation of the CpG island near the G4C2 repeat in ALS with a C9orf72 expansion. Am J Hum Genet (2013) 1.97
Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic. Cell Rep (2013) 1.96
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
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A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43. Hum Mol Genet (2011) 1.47
Huntington's disease protein contributes to RNA-mediated gene silencing through association with Argonaute and P bodies. Proc Natl Acad Sci U S A (2008) 1.35
Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72. Acta Neuropathol Commun (2013) 1.32
Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype. Neurobiol Aging (2012) 1.30
RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations. Hum Mol Genet (2012) 1.19
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c9RAN translation: a potential therapeutic target for the treatment of amyotrophic lateral sclerosis and frontotemporal dementia. Expert Opin Ther Targets (2013) 0.85