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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 
TranscriptomicsRNA-seq of primary murine (C57BL/6J) LSK cells overexpressing miR-10a or miR-335
In this experiment, we want to assess the effect of a lentiviral miR-10a and miR-335 overexpression on the transcriptome of murine LSK (Lin-,Sca-1+,c-Kit+) cells. Primary LSK cells were transduced with lentiviral miRNA overexpression constructs (control: GFP overexpression) and sorted for transduced...
ORGANISM(S): Mus musculus 
2018-06-01 | E-MTAB-6496 | biostudies-arrayexpress
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 
UnknownC9orf72 protein quality control by UBR5-mediated heterotypic ubiquitin chains.
Not available
S-EPMC10398660 | biostudies-literature
UnknownRole of Septin cytoskeleton in spine morphogenesis and dendrite development in neurons.
Not available
S-EPMC2194646 | biostudies-literature
UnknownIdentification and characterization of neuronal mitogen-activated protein kinase substrates using a specific phosphomotif antibody.
Not available
S-EPMC2667352 | biostudies-literature
UnknownProteomics and C9orf72 neuropathology identify ribosomes as poly-GR/PR interactors driving toxicity.
Not available
S-EPMC6238541 | biostudies-literature
UnknownComparative CRISPRi screens reveal a human stem cell dependence on mRNA translation-coupled quality control.
Not available
S-EPMC12527931 | biostudies-literature
UnknownPresenilin-1 affects trafficking and processing of betaAPP and is targeted in a complex with nicastrin to the plasma membrane.
Not available
S-EPMC2173840 | biostudies-literature
UnknownMicroRNA-125b induces tau hyperphosphorylation and cognitive deficits in Alzheimer's disease.
Not available
S-EPMC4194100 | biostudies-literature
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