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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Frontotemporal dementia is a group of early onset dementia syndromes linked to underlying frontotemporal lobar degeneration (FTLD) pathology that can be classified based on the formation of abnormal protein aggregates involving tau and two RNA binding proteins, TDP-43 and FUS.
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30355151 |
2019 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Numerous reports have demonstrated by pathological and genetic analysis that FUS is associated with a variety of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration (FTLD), and polyglutamine diseases.
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31022909 |
2019 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
The neuropathological correlate of FTD is frontotemporal lobar degeneration (FTLD), characterized by tau-, TDP-43-, and FUS-immunoreactive neuronal inclusions.
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30774737 |
2019 |
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Frontotemporal Lobar Degeneration
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0.100 |
GeneticVariation
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disease |
BEFREE |
To characterize the topography of white matter pathology in neuronal intermediate filament inclusion disease (NIFID), a rare subtype of frontotemporal lobar degeneration (FTLD) with "fused in sarcoma" (FUS)-immunoreactive inclusions.
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29956645 |
2019 |
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Frontotemporal Lobar Degeneration
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0.100 |
AlteredExpression
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disease |
BEFREE |
Severe motor dysfunction and early lethality of mice with expression above this level prevent their use for studies of FTLD-related pathology caused by expression of this form of FUS.
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31437340 |
2019 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
The FTLD-FUS subgroup is defined by the presence of the fused in sarcoma protein (FUS) in pathological inclusions.
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30755280 |
2019 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
FTD usually belongs to the frontotemporal lobar degeneration (FTLD) disease group, and its familial forms are dominantly inherited and linked to a group of genes relevant to frontal and temporal brain pathology, such as MAPT, GRN, C9ORF72, TARDBP, CHMP2B, VCP, and FUS.
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29578490 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Fused in sarcoma (FUS) is an RNA binding protein that is involved in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS).
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28842245 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Thus, to facilitate development of early disease markers and/or therapeutic targets of FTLD/ALS it is critical that the functions of FUS and its downstream pathways are unraveled.
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29774215 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Indeed, significant hypomethylation, which occurs in FUS-associated frontotemporal lobar degeneration (FTLD), induces FUS condensation into stable intermolecular β-sheet-rich hydrogels that disrupt RNP granule function and impair new protein synthesis in neuron terminals.
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29677515 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
GeneticVariation
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disease |
BEFREE |
To gain better insight into the molecular interactions underlying this process, we investigated FUS, which is mutated and aggregated in both ALS and FTLD.
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30021151 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
GeneticVariation
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disease |
BEFREE |
Using FUS as an example, this review examines the biophysics of this physiological process, and reports on how mutations and changes in post-translational state alter phase behaviour, and lead to neurodegenerative diseases such as amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
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29723523 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Moreover, FUS is associated with abnormalities in post-synaptic formation, which can be an early disease marker of FTLD.
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30050404 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
AlteredExpression
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disease |
BEFREE |
NPM-hMLF1 fusion protein suppresses defects of a Drosophila FTLD model expressing the human FUS gene.
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30050143 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
These FUS protein aggregates are known as pathological hallmarks in a subset of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) cases.
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30002616 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
FTLD classification distinguishes three main neuropathological groups: FTLD-tau, FTLD-TDP, and FTLD-FUS.
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29758948 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
FUS (fused in sarcoma) proteinopathy is a group of neurodegenerative diseases characterized by the formation of inclusion bodies containing the FUS protein, including frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
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30249657 |
2018 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Patients with FTLD were distributed between FTLD-tau (34 patients: 10 corticobasal degeneration, nine progressive supranuclear palsy, eight Pick's disease, three frontotemporal dementia with parkinsonism associated with chromosome 17, three unclassifiable tauopathy, and one argyrophilic grain disease); FTLD-TDP (55 patients: nine type A including one with motor neuron disease, 27 type B including 21 with motor neuron disease, eight type C with right temporal lobe presentations, and 11 unclassifiable including eight with motor neuron disease), FTLD-FUS (eight patients), and one patient with FTLD-ubiquitin proteasome system positive inclusions (FTLD-UPS) that stained negatively for tau, TDP-43, and FUS.
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29053860 |
2017 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Mutations within TDP-43 or FUS are themselves neuropathogenic in ALS and some cases of FTLD.
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28358904 |
2017 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Altered Tau Isoform Ratio Caused by Loss of FUS and SFPQ Function Leads to FTLD-like Phenotypes.
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28147269 |
2017 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Evidence suggests that cytoplasmic mislocalization of nuclear proteins such as transactive response DNA-binding protein 43 (TDP-43) and fused in sarcoma (FUS) may be associated with neurotoxicity in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration.
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28453527 |
2017 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Rapidly progressive Fronto-temporal dementia (FTD) associated with Frontotemporal lobar degeneration (FTLD) in the presence of Fused in Sarcoma (FUS) protein: a rare, sporadic, and aggressive form of FTD.
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28660843 |
2017 |
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Frontotemporal Lobar Degeneration
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0.100 |
GeneticVariation
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disease |
BEFREE |
Mutations in TDP-43 and FUS genes are linked to clinical ALS rather than FTLD (with or without ALS), suggesting that clinical ALS may be a disorder of RNA metabolism.
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28100023 |
2017 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Hence, the spatial patterns of the NCI were compared in three molecular subtypes of FTLD: (1) two variants of FTLD-tau, viz. cortico-basal degeneration (CBD) and Pick's disease (PiD), (2) FTLD with transactive response (TAR) DNA-binding protein 43(TDP-43)-immunoreactive inclusions (FTLD-TDP), and (3) FTLD with 'fused in sarcoma' (FUS)-immunoreactive inclusions (FTLD-FUS).
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28984110 |
2017 |
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Frontotemporal Lobar Degeneration
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0.100 |
Biomarker
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disease |
BEFREE |
Finally, abnormal spine maturation and FTLD-like behavioral deficits in FUS-knockout mice were ameliorated by SynGAP α2.
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28954225 |
2017 |