ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

scientific article published on 4 February 2016

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function is …
instance of (P31):
scholarly articleQ13442814

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P819ADS bibcode2016NatCo...710465S
P356DOI10.1038/NCOMMS10465
P932PMC publication ID4742863
P698PubMed publication ID26842965
P5875ResearchGate publication ID293015007

P2093author name stringLei Lu
George Z Mentis
Neil A Shneider
Aarti Sharma
Adriana Nemes
Sara Ebrahimi Nasrabady
Juan Carlos Tapia
Monica Mendelsohn
Alexander K Lyashchenko
Margot Elmaleh
P2860cites workNovel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis.Q51774971
Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death.Q52537887
Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTFQ60220472
Differential subcellular localization of tubulin and the microtubule-associated protein MAP2 in brain tissue as revealed by immunocytochemistry with monoclonal hybridoma antibodiesQ71277329
Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and manQ75310510
Inducible nitric oxide synthase up-regulation in a transgenic mouse model of familial amyotrophic lateral sclerosisQ77816092
A compensatory subpopulation of motor neurons in a mouse model of amyotrophic lateral sclerosisQ81011294
An ALS-associated mutation in the FUS 3'-UTR disrupts a microRNA-FUS regulatory circuitryQ87367862
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALSQ24629495
ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear importQ24630100
A developmental switch in the response of DRG neurons to ETS transcription factor signalingQ24791095
FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosisQ27339258
FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degenerationQ27342067
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosisQ28131672
Proteomic analysis of NMDA receptor-adhesion protein signaling complexesQ28138918
Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseasesQ28139487
Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosisQ28236796
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6Q28236805
Deletion of the developmentally essential gene ATR in adult mice leads to age-related phenotypes and stem cell lossQ28274165
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementiaQ28294001
Delocalization of the multifunctional RNA splicing factor TLS/FUS in hippocampal neurones: exclusion from the nucleus and accumulation in dendritic granules and spine headsQ28582163
Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogelsQ29614781
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosisQ29616311
Protamine-Cre recombinase transgenes efficiently recombine target sequences in the male germ line of mice, but not in embryonic stem cellsQ29618298
Onset and progression in inherited ALS determined by motor neurons and microgliaQ29619515
Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashionQ30532680
Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse modelsQ30572264
Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutationsQ30573122
Microglia induce motor neuron death via the classical NF-κB pathway in amyotrophic lateral sclerosisQ30575638
Phosphorylation-regulated binding of RNA polymerase II to fibrous polymers of low-complexity domainsQ30577537
Fus gene mutations in familial and sporadic amyotrophic lateral sclerosisQ34265540
Functions of FUS/TLS from DNA repair to stress response: implications for ALS.Q34305789
Activity-dependent FUS dysregulation disrupts synaptic homeostasisQ34480824
TDP-43 N terminus encodes a novel ubiquitin-like fold and its unfolded form in equilibrium that can be shifted by binding to ssDNAQ34831850
Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cellsQ35695216
Immunologic reactions in amyotrophic lateral sclerosis brain and spinal cord tissueQ35830662
Widespread distribution of the major polypeptide component of MAP 1 (microtubule-associated protein 1) in the nervous systemQ36209567
Light and electron microscope localization of the microtubule-associated tau protein in rat brainQ36433582
Light and electron microscopic studies of the distribution of microtubule-associated protein 2 in rat brain: a difference between dendritic and axonal cytoskeletonsQ36626479
Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAsQ36650975
High-contrast en bloc staining of neuronal tissue for field emission scanning electron microscopyQ36981939
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
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyondQ37642283
Motor neuron diversity in development and diseaseQ37724333
Clinical genetics of amyotrophic lateral sclerosis: what do we really know?Q37944428
Axonal mRNA localization and local protein synthesis in nervous system assembly, maintenance and repairQ38002309
The role of FUS gene variants in neurodegenerative diseasesQ38212812
The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span.Q38732625
Intranuclear aggregation of mutant FUS/TLS as a molecular pathomechanism of amyotrophic lateral sclerosisQ39054098
ALS-associated mutations in FUS disrupt the axonal distribution and function of SMN.Q39150934
Cell-free formation of RNA granules: bound RNAs identify features and components of cellular assemblies.Q39349822
Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis.Q40165089
Identification and characterization of the nuclear localization/retention signal in the EWS proto-oncoproteinQ40233283
Familial amyotrophic lateral sclerosis with a mutation in exon 4 of the Cu/Zn superoxide dismutase gene: pathological and immunocytochemical changesQ41187994
Amyotrophic lateral sclerosis: macro-EMG and twitch forces of single motor unitsQ44481993
P525L FUS mutation is consistently associated with a severe form of juvenile amyotrophic lateral sclerosis.Q45894689
ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions.Q46135324
Differentiation of mouse embryonic stem cells to spinal motor neuronsQ46390435
The RNA binding protein TLS is translocated to dendritic spines by mGluR5 activation and regulates spine morphology.Q46409672
Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS.Q47951637
Mutations in the 3' untranslated region of FUS causing FUS overexpression are associated with amyotrophic lateral sclerosis.Q48021519
AMPA receptors regulate dynamic equilibrium of presynaptic terminals in mature hippocampal networksQ48337086
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P6216copyright statuscopyrightedQ50423863
P4510describes a project that usesImageJQ1659584
P407language of work or nameEnglishQ1860
P921main subjectamyotrophic lateral sclerosisQ206901
P304page(s)10465
P577publication date2016-02-04
P1433published inNature CommunicationsQ573880
P1476titleALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
P478volume7

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cites work (P2860)
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