Abl kinase-mediated FUS Tyr526 phosphorylation alters nucleocytoplasmic FUS localization in FTLD-FUS [PDF]
Brain, 2023 Abstract Nuclear to cytoplasmic mislocalization and aggregation of multiple RNA-binding proteins (RBPs), including FUS, are the main neuropathological features of the majority of cases of amyotrophic lateral sclerosis (ALS) and frontotemporal lobular degeneration (FTLD).
Helena Motaln +6 more
openaire +4 more sources
Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation [PDF]
Molecular Neurodegeneration, 2021 Mutations in FUS, an RNA-binding protein involved in multiple steps of RNA metabolism, are associated with the most severe forms of amyotrophic lateral sclerosis (ALS).
Inmaculada Sanjuan-Ruiz +14 more
doaj +2 more sources
ALS-associated FUS mutations result in compromised FUS alternative splicing and autoregulation. [PDF]
PLoS Genetics, 2013 The gene encoding a DNA/RNA binding protein FUS/TLS is frequently mutated in amyotrophic lateral sclerosis (ALS). Mutations commonly affect its carboxy-terminal nuclear localization signal, resulting in varying deficiencies of FUS nuclear localization ...
Yueqin Zhou +4 more
doaj +3 more sources
ALS-FUS pathology revisited: singleton FUS mutations and an unusual case with both a FUS and TARDBP mutation [PDF]
Acta Neuropathologica Communications, 2015 Mutations in the FUS gene have been shown to be a rare cause of amyotrophic lateral sclerosis (ALS-FUS) and whilst well documented clinically and genetically there have been relatively few neuropathological studies.Recent work suggested a possible correlation between pathological features such as frequency of basophilic inclusions in neurons and rate ...
King, A. +7 more
openaire +6 more sources
Frameshift peptides alter the properties of truncated FUS proteins in ALS-FUS [PDF]
Molecular Brain, 2020 Mutations in the FUS gene cause a subset of ALS cases (ALS-FUS). The majority of FUS mutations are missense mutations affecting the nuclear localisation signal (NLS) of FUS.
Haiyan An +3 more
doaj +3 more sources
FOB microscopy - HeLa P525L GFP-FUS [PDF]
, 2021 Fluorescence, optical diffraction tomography and Brillouin microscopy data of HeLa P525L GFP-FUS cells under control conditions, after arsenite treatment, and after arsenite treatment and chemical fixation.
Jochen Guck (7429550) +3 more
core +1 more source
Synaptic FUS accumulation triggers early misregulation of synaptic RNAs in a mouse model of ALS [PDF]
, 2021 International audienceMutations disrupting the nuclear localization of the RNA-binding protein FUS characterize a subset of amyotrophic lateral sclerosis patients (ALS-FUS).
Pérez-Berlanga, Manuela +11 more
core +1 more source
FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA [PDF]
, 2012 Mutations in the RNA binding protein FUS cause amyotrophic lateral sclerosis (ALS), a fatal adult motor neuron disease. Decreased expression of SMN causes the fatal childhood motor neuron disorder spinal muscular atrophy (SMA).
Das, Rita +56 more
core +1 more source
Current Directions in Biomedical Engineering, 2020 Employing sonosensitive nanoparticles as carriers of active pharmaceutical ingredients emerges in ultrasonic Drug Delivery. Drug release can be initiated by focused ultrasound via the effect of inertial cavitation in certain target areas of particle ...
George Benedikt +7 more
doaj +1 more source
A Genetic Screen for Human Genes Suppressing FUS Induced Toxicity in Yeast
G3: Genes, Genomes, Genetics, 2020 FUS is a nucleic acid binding protein that, when mutated, cause a subset of familial amyotrophic lateral sclerosis (ALS). Expression of FUS in yeast recapitulates several pathological features of the disease-causing mutant proteins, including nuclear to ...
Elliott Hayden +5 more
doaj +1 more source