|
1
|
Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS.
|
Neuron
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2013
|
5.91
|
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2
|
Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity.
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Proc Natl Acad Sci U S A
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2009
|
3.65
|
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3
|
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice.
|
J Neurosci
|
2010
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2.60
|
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4
|
Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons.
|
Acta Neuropathol
|
2013
|
2.26
|
|
5
|
Autophagic-lysosomal perturbation enhances tau aggregation in transfectants with induced wild-type tau expression.
|
Eur J Neurosci
|
2008
|
1.54
|
|
6
|
Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress.
|
Acta Neuropathol
|
2014
|
1.54
|
|
7
|
Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood.
|
Acta Neuropathol
|
2013
|
1.52
|
|
8
|
Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia.
|
Acta Neuropathol
|
2013
|
1.41
|
|
9
|
Misregulation of human sortilin splicing leads to the generation of a nonfunctional progranulin receptor.
|
Proc Natl Acad Sci U S A
|
2012
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1.07
|
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10
|
RNA-mediated toxicity in neurodegenerative disease.
|
Mol Cell Neurosci
|
2012
|
1.07
|
|
11
|
Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragments.
|
Mol Neurodegener
|
2010
|
1.06
|
|
12
|
Acetylation of the KXGS motifs in tau is a critical determinant in modulation of tau aggregation and clearance.
|
Hum Mol Genet
|
2013
|
1.05
|
|
13
|
The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation.
|
Hum Mol Genet
|
2013
|
1.02
|
|
14
|
Loss of HDAC6, a novel CHIP substrate, alleviates abnormal tau accumulation.
|
Hum Mol Genet
|
2012
|
1.00
|
|
15
|
Targeted manipulation of the sortilin-progranulin axis rescues progranulin haploinsufficiency.
|
Hum Mol Genet
|
2013
|
0.93
|
|
16
|
An alternative Ca2+-dependent mechanism of neuroprotection by the metalloporphyrin class of superoxide dismutase mimetics.
|
FASEB J
|
2005
|
0.91
|
|
17
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Progressive amnestic dementia, hippocampal sclerosis, and mutation in C9ORF72.
|
Acta Neuropathol
|
2013
|
0.89
|
|
18
|
c9RAN translation: a potential therapeutic target for the treatment of amyotrophic lateral sclerosis and frontotemporal dementia.
|
Expert Opin Ther Targets
|
2013
|
0.85
|
|
19
|
Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy.
|
Acta Neuropathol
|
2013
|
0.83
|
|
20
|
Concentration-dependent effects of proteasomal inhibition on tau processing in a cellular model of tauopathy.
|
Int J Clin Exp Pathol
|
2009
|
0.83
|
|
21
|
Discovery of a Biomarker and Lead Small Molecules to Target r(GGGGCC)-Associated Defects in c9FTD/ALS.
|
Neuron
|
2014
|
0.75
|