Search Results - "Farny, Daniel"
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Gel‐like inclusions of C‐terminal fragments of TDP‐43 sequester stalled proteasomes in neurons
Published in EMBO reports (07-06-2022)“…Aggregation of the multifunctional RNA‐binding protein TDP‐43 defines large subgroups of amyotrophic lateral sclerosis and frontotemporal dementia and…”
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Active poly‐GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model
Published in EMBO molecular medicine (07-02-2020)“…The C9orf72 repeat expansion is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and/or frontotemporal dementia (FTD). Non‐canonical…”
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Proteomics and C9orf72 neuropathology identify ribosomes as poly-GR/PR interactors driving toxicity
Published in Life science alliance (01-05-2018)“…Frontotemporal dementia and amyotrophic lateral sclerosis patients with mutation show cytoplasmic poly-GR and poly-PR aggregates. Short poly-(Gly-Arg) and…”
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Antibodies inhibit transmission and aggregation of C9orf72 poly‐GA dipeptide repeat proteins
Published in EMBO molecular medicine (01-05-2017)“…Cell‐to‐cell transmission of protein aggregates is an emerging theme in neurodegenerative disease. Here, we analyze the dipeptide repeat (DPR) proteins that…”
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Targeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo
Published in Acta neuropathologica communications (11-07-2023)“…Cytoplasmic aggregation and concomitant nuclear clearance of the RNA-binding protein TDP-43 are found in ~ 90% of cases of amyotrophic lateral sclerosis and ~…”
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Cell‐to‐cell transmission of C9orf72 poly‐(Gly‐Ala) triggers key features of ALS/FTD
Published in The EMBO journal (15-04-2020)“…The C9orf72 repeat expansion causes amyotrophic lateral sclerosis and frontotemporal dementia, but the poor correlation between C9orf72 ‐specific pathology and…”
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Author Correction: Gel-like inclusions of C-terminal fragments of TDP-43 sequester stalled proteasomes in neurons
Published in EMBO reports (01-07-2024)Get full text
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Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS
Published in Acta neuropathologica (01-08-2020)“…Expansion of a (G 4 C 2 ) n repeat in C9orf72 causes amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), but the link of the five…”
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Drug screen in iPSC-Neurons identifies nucleoside analogs as inhibitors of (G 4 C 2 ) n expression in C9orf72 ALS/FTD
Published in Cell reports (Cambridge) (07-06-2022)“…An intronic (G C ) expansion in C9orf72 causes amyotrophic lateral sclerosis and frontotemporal dementia primarily through gain-of-function mechanisms: the…”
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