|
Amyotrophic Lateral Sclerosis
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
Here, we identified that Cdc48 and Ubx3, a Cdc48 co-factor implicated in endocytic function, regulates the turnover and toxicity of TDP-43 and FUS expressed in <i>S. cerevisiae</i> Cdc48 physically interacts and co-localizes with TDP-43, as does VCP in ALS patient tissue.
|
31767634 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
The RNA-binding proteins TDP-43 and FUS are tied as the third leading known genetic cause for amyotrophic lateral sclerosis (ALS), and TDP-43 proteopathies are found in nearly all ALS patients.
|
31693373 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
We reveal two distinct mechanisms underpinning potentially disparate pathogenic pathways of ALS-linked FUS mutants.
|
31630970 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
In addition, those pathological neurofilament accumulations are known in α-synuclein in Parkinson's disease (PD), Aβ and tau in Alzheimer's disease (AD), polyglutamine in CAG trinucleotide repeat disorders, superoxide dismutase 1 (SOD1), TAR DNA-binding protein 43 (TDP43), neuronal FUS proteins, optineurin (OPTN), ubiquilin 2 (UBQLN2), and dipeptide repeat protein (DRP) in amyotrophic lateral sclerosis (ALS).
|
31820696 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
C9orf72, SOD1, TARDBP, and FUS are noted as the most common ALS genes; however, mutations of these genes explain <10% of sALS cases.
|
31060816 |
2020 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Here, we assessed vascular integrity <i>in vivo</i> throughout different disease stages, and investigated whether ANG treatment reverses vascular regression and prolongs motor neuron survival in the FUS (1-359) mouse model of ALS.
|
31383794 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
This review summarizes recent studies on FUS self-assembling, including both aggregation and LLPS as well as their relationship with the pathology of ALS, FTLD, and other neurodegenerative diseases.
|
31022909 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
We find that rabies virus (RABV) spreads ALS phenotypes, including the formation of stress granules (SGs) with aberrant composition due to increased levels of FUS protein, as well as neurodegeneration and reduced restriction activity by FUS mutations.
|
31695598 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
Combined fused in sarcoma-positive (FUS+) basophilic inclusion body disease and atypical tauopathy presenting with an amyotrophic lateral sclerosis/motor neurone disease (ALS/MND)-plus phenotype.
|
30659642 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
Thus, FUS regulates acetylcholine receptor gene expression in subsynaptic myonuclei, and muscle-intrinsic toxicity of ALS mutant FUS may contribute to dying-back motor neuronopathy.
|
31591561 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Current work indicates that FUS mutations may incur gain-of-toxic functions to drive ALS pathogenesis.
|
31509188 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
We report the largest genotype-phenotype correlation of FUS-ALS, which enables a careful prediction of the clinical course in newly diagnosed patients.
|
31682085 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Broad expression within the central nervous system of either wild-type or two ALS-linked human FUS mutants produces progressive motor phenotypes accompanied by characteristic ALS-like pathology.
|
31230528 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
Caz is a Drosophila homologue of FUS, which is one of the genes causing amyotrophic lateral sclerosis (ALS).
|
30578769 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
C9orf72, SOD1, TDP-43 and FUS are ranked as the four major genes causing familial ALS.
|
31822699 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
A unified mechanism for LLPS of ALS/FTLD-causing FUS as well as its modulation by ATP and oligonucleic acids.
|
31188823 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
In summary, we report widespread mislocalization of the FUS protein in ALS and propose a putative underlying mechanism for this process.
|
31368485 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
The mechanistic link between altered FUS levels and ALS-related neurodegeneration is far to be elucidated, as well as the consequences of elevated FUS levels in the modulation of the inflammatory response sustained by glial cells, a well-recognized player in ALS progression.
|
30872738 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
To address these questions, a quantitative proteomics approach was used to identify proteins that evade stress-induced translational repression in arsenite-treated cells expressing either wild-type or amyotrophic lateral sclerosis (ALS)-linked mutant FUS.
|
30806671 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
In the spinal cord of FUS ALS patients, ELAVL4 represents a neural-specific component of FUS-positive cytoplasmic aggregates, whereas in sporadic patients it co-localizes with phosphorylated TDP-43-positive inclusions.
|
31242416 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
Previously, we engineered potentiated Hsp104 variants to suppress the proteotoxicity, aggregation, and mislocalization of FUS and other proteins that aggregate in ALS/FTD and Parkinson's disease.
|
31171724 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
Biomarker
|
disease |
BEFREE |
We show that FUS' tendency to aggregate is normally buffered by interacting RBPs, but this buffering is lost when FUS mislocalizes to the cytoplasm due to ALS mutations.
|
30937520 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
The commonality of those phenotypes was further confirmed with other ALS causative mutation than FUS.
|
31262712 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
By comparing patient-derived MNs and their isogenic controls, we show that ALS-causing mutations in FUS did not affect glycolytic or mitochondrial energy metabolism of human MNs in vitro.
|
31515480 |
2019 |
|
Amyotrophic Lateral Sclerosis
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Mutations in the FUS gene cause amyotrophic lateral sclerosis (ALS-FUS).
|
30642400 |
2019 |