Importance of Functional Loss of FUS in FTLD/ALS.

scientific article published on 3 May 2018

Importance of Functional Loss of FUS in FTLD/ALS. is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.3389/FMOLB.2018.00044
P932PMC publication ID5943504
P698PubMed publication ID29774215

P50authorGen SobueQ67219868
P2093author name stringShinsuke Ishigaki
P2860cites workFusion of CHOP to a novel RNA-binding protein in human myxoid liposarcomaQ24309359
hTAF(II)68, a novel RNA/ssDNA-binding protein with homology to the pro-oncoproteins TLS/FUS and EWS is associated with both TFIID and RNA polymerase IIQ24562004
Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcriptionQ24646744
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FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosisQ27339258
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Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6Q28236805
TLS facilitates transport of mRNA encoding an actin-stabilizing protein to dendritic spinesQ28284342
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Onset and progression in inherited ALS determined by motor neurons and microgliaQ29619515
FUS binds the CTD of RNA polymerase II and regulates its phosphorylation at Ser2.Q30456334
Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a reviewQ33726688
Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutationsQ34099212
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Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementiaQ34125553
Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusionsQ34183099
Knockdown of the Drosophila fused in sarcoma (FUS) homologue causes deficient locomotive behavior and shortening of motoneuron terminal branches.Q34314477
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Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death.Q52537887
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A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation.Q53368534
Liquid-liquid phase separation of the microtubule-binding repeats of the Alzheimer-related protein Tau.Q38619152
The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span.Q38732625
Dendritic Spine Pathology in Neurodegenerative DiseasesQ38747180
Position-specific binding of FUS to nascent RNA regulates mRNA lengthQ38872764
Liquid-liquid phase separation in cellular signaling systemsQ38934732
Altered Tau Isoform Ratio Caused by Loss of FUS and SFPQ Function Leads to FTLD-like Phenotypes.Q39095704
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PINK1 and Parkin are genetic modifiers for FUS-induced neurodegeneration.Q39235670
Intracellular localization and splicing regulation of FUS/TLS are variably affected by amyotrophic lateral sclerosis-linked mutationsQ39627330
Axonal transport defects are a common phenotype in Drosophila models of ALS.Q39774347
FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosisQ39927199
Silencing of FUS in the common marmoset (Callithrix jacchus) brain via stereotaxic injection of an adeno-associated virus encoding shRNA.Q40070020
Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule DynamicsQ40287573
Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neuronsQ41644415
FUS regulates AMPA receptor function and FTLD/ALS-associated behaviour via GluA1 mRNA stabilizationQ41950782
Widespread binding of FUS along nascent RNA regulates alternative splicing in the brainQ41955381
Loss of fused in sarcoma (FUS) promotes pathological Tau splicingQ42138048
FUS regulates genes coding for RNA-binding proteins in neurons by binding to their highly conserved intronsQ42146718
Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptionsQ42246467
HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patientsQ42261802
Non-nuclear Pool of Splicing Factor SFPQ Regulates Axonal Transcripts Required for Normal Motor Development.Q42347699
Phosphorylation of the FUS low-complexity domain disrupts phase separation, aggregation, and toxicity.Q42378053
Cellular tau pathology and immunohistochemical study of tau isoforms in sporadic tauopathiesQ42503599
TDP-43 loss-of-function causes neuronal loss due to defective steroid receptor-mediated gene program switching in DrosophilaQ43593822
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3'UTR Length-Dependent Control of SynGAP Isoform α2 mRNA by FUS and ELAV-like Proteins Promotes Dendritic Spine Maturation and Cognitive FunctionQ47735809
Structure of FUS Protein Fibrils and Its Relevance to Self-Assembly and Phase Separation of Low-Complexity DomainsQ47752760
Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.Q47946104
Mutation-specific functional impairments in distinct tau isoforms of hereditary FTDP-17.Q48338403
Activity-dependent FUS dysregulation disrupts synaptic homeostasisQ34480824
Tau mislocalization to dendritic spines mediates synaptic dysfunction independently of neurodegenerationQ34518577
Male sterility and enhanced radiation sensitivity in TLS(-/-) miceQ34662602
ALS-associated FUS mutations result in compromised FUS alternative splicing and autoregulationQ35034350
ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP.Q35114564
Familial behavioral variant frontotemporal dementia associated with astrocyte-predominant tauopathyQ35196395
Differential regulation of microtubule dynamics by three- and four-repeat tau: implications for the onset of neurodegenerative diseaseQ35234889
Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutationQ35869162
TLS/FUS (translocated in liposarcoma/fused in sarcoma) regulates target gene transcription via single-stranded DNA response elementsQ35924992
ALS mutations in TLS/FUS disrupt target gene expressionQ36032446
ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule FunctionQ36320469
Super-Resolution Microscopy Reveals Presynaptic Localization of the ALS/FTD Related Protein FUS in Hippocampal NeuronsQ36453024
The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrumQ36485513
ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of functionQ36548825
Spliceosome integrity is defective in the motor neuron diseases ALS and SMA.Q36602121
Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAsQ36650975
Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice.Q36677109
Establishment of In Vitro FUS-Associated Familial Amyotrophic Lateral Sclerosis Model Using Human Induced Pluripotent Stem Cells.Q36805034
Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron lossQ36906420
The LC Domain of hnRNPA2 Adopts Similar Conformations in Hydrogel Polymers, Liquid-like Droplets, and NucleiQ36934790
Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses.Q37072993
FUS-regulated region- and cell-type-specific transcriptome is associated with cell selectivity in ALS/FTLD.Q37080289
The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neuronsQ37364300
The RNA-binding protein Fus directs translation of localized mRNAs in APC-RNP granulesQ37379010
Rethinking ALS: the FUS about TDP-43.Q37419912
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defectsQ37609639
FUS affects circular RNA expression in murine embryonic stem cell-derived motor neurons.Q37736433
TDP-43 and FUS/TLS: sending a complex message about messenger RNA in amyotrophic lateral sclerosis?Q37910303
The changing scene of amyotrophic lateral sclerosisQ38086771
FUS-mediated alternative splicing in the nervous system: consequences for ALS and FTLD.Q38131381
Lower motor neuron involvement in TAR DNA-binding protein of 43 kDa-related frontotemporal lobar degeneration and amyotrophic lateral sclerosisQ38174758
The phenotypic variability of amyotrophic lateral sclerosisQ38259758
Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS.Q38314794
TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like cells.Q38326803
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P6216copyright statuscopyrightedQ50423863
P921main subjectamyotrophic lateral sclerosisQ206901
P304page(s)44
P577publication date2018-05-03
P1433published inFrontiers in molecular biosciencesQ27726420
P1476titleImportance of Functional Loss of FUS in FTLD/ALS.
P478volume5

Reverse relations

cites work (P2860)
Q90451856Differential subcellular expression of P525LFUS as a putative biomarker for ALS phenoconversion
Q64096028Increased FUS levels in astrocytes leads to astrocyte and microglia activation and neuronal death
Q91946029Role of RNA Binding Proteins with prion-like domains in muscle and neuromuscular diseases
Q64999275Synaptic FUS Localization During Motoneuron Development and Its Accumulation in Human ALS Synapses.
Q89963639The Overlapping Genetics of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
Q93159410Ubiquilin 2 modulates ALS/FTD-linked FUS-RNA complex dynamics and stress granule formation

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