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ProteomicsTranslation deficits drive C9orf72 associated poly-GR/PR toxicity
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) patients with the C9orf72 mutation show predominantly cytoplasmic aggregates of poly-GR and poly-PR proteins that are acutely toxic in various model systems. To identify the molecular mediators of neurotoxicity of poly-GR/PR, we a...
ORGANISM(S): Homo sapiens (Human) Rattus norvegicus (Rat) 
2018-06-27 | PXD008691 | Pride
Ftld Proteomics Stress granules Als Ribosome Nucleolus Dpr proteins Interactome C9orf72 Translation 
ProteomicsTDP-43 loss of function inhibits endosomal trafficking and alters trophic signaling in neurons - Surface labelling
Nuclear clearance of TDP-43 into cytoplasmic aggregates is a key driver of neurodegeneration in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), but the mechanisms are unclear. Here, we show that TDP-43 knockdown specifically reduces number and motility of RAB11-posi...
ORGANISM(S): Mus musculus (Mouse) 
2016-09-29 | PXD004744 | Pride
Ftld Recycling endosomes Tdp-43 Als Erbb4 Surface labelling 
UnknownTargeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo.
Not available
S-EPMC10334564 | biostudies-literature
UnknownCell-to-cell transmission of C9orf72 poly-(Gly-Ala) triggers key features of ALS/FTD
Not available
S-SCDT-EMBOJ-2019-102811 | biostudies-other
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