scholarly article | Q13442814 |
P356 | DOI | 10.1093/BRAIN/AWW161 |
P698 | PubMed publication ID | 27368346 |
P50 | author | Daisuke Ito | Q30430138 |
P2093 | author name string | Norihiro Suzuki | |
Takuya Yagi | |||
Yoshihiro Nihei | |||
Gen Shiihashi | |||
Taeko Ebine | |||
P2860 | cites work | Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease | Q24628685 |
ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import | Q24630100 | ||
Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS | Q24634807 | ||
A new subtype of frontotemporal lobar degeneration with FUS pathology | Q24647697 | ||
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis | Q28131672 | ||
Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis | Q28236796 | ||
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6 | Q28236805 | ||
Mutations in the tail domain of DYNC1H1 cause dominant spinal muscular atrophy | Q28263116 | ||
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis | Q28270779 | ||
Microglia-specific localisation of a novel calcium binding protein, Iba1 | Q28273977 | ||
Role of axonal transport in neurodegenerative diseases | Q28284096 | ||
Age-dependent deterioration of nuclear pore complexes causes a loss of nuclear integrity in postmitotic cells | Q28572651 | ||
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis | Q29615597 | ||
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport. | Q29871482 | ||
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration | Q30493749 | ||
Deficits in axonal transport precede ALS symptoms in vivo | Q30497554 | ||
Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion | Q30532680 | ||
Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations | Q30573122 | ||
FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration | Q33922335 | ||
Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules | Q34308339 | ||
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport | Q34491033 | ||
ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP. | Q35114564 | ||
Genetic background effects on disease onset and lifespan of the mutant dynactin p150Glued mouse model of motor neuron disease | Q35170908 | ||
Exome sequencing identifies FUS mutations as a cause of essential tremor | Q36152927 | ||
FUS-SMN protein interactions link the motor neuron diseases ALS and SMA | Q36354418 | ||
ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function | Q36548825 | ||
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-mRNAs | Q36650975 | ||
Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS. | Q36795394 | ||
Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss | Q36906420 | ||
ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules | Q36908224 | ||
ER stress and unfolded protein response in amyotrophic lateral sclerosis | Q37382212 | ||
Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43. | Q37451385 | ||
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects | Q37609639 | ||
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration | Q37733168 | ||
Effect of genetic background on phenotype variability in transgenic mouse models of amyotrophic lateral sclerosis: a window of opportunity in the search for genetic modifiers | Q37829424 | ||
The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene | Q38493216 | ||
ALS-associated mutations in FUS disrupt the axonal distribution and function of SMN. | Q39150934 | ||
FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis | Q39927199 | ||
FUS regulates AMPA receptor function and FTLD/ALS-associated behaviour via GluA1 mRNA stabilization | Q41950782 | ||
Loss of fused in sarcoma (FUS) promotes pathological Tau splicing | Q42138048 | ||
Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43. | Q42940304 | ||
Decreased number of Gemini of coiled bodies and U12 snRNA level in amyotrophic lateral sclerosis. | Q50932808 | ||
Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration. | Q52546347 | ||
Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS. | Q52607544 | ||
Mutations in dynein link motor neuron degeneration to defects in retrograde transport | Q55984322 | ||
Characterization of inclusion bodies with cytoprotective properties formed by seipinopathy-linked mutant seipin | Q56985709 | ||
N88S seipin mutant transgenic mice develop features of seipinopathy/BSCL2-related motor neuron disease via endoplasmic reticulum stress | Q56985760 | ||
P433 | issue | Pt 9 | |
P407 | language of work or name | English | Q1860 |
P921 | main subject | amyotrophic lateral sclerosis | Q206901 |
P304 | page(s) | 2380-2394 | |
P577 | publication date | 2016-06-30 | |
P1433 | published in | Brain | Q897386 |
P1476 | title | Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice | |
P478 | volume | 139 |
Q99547009 | ALS Genetics: Gains, Losses, and Implications for Future Therapies |
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Q57813809 | ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS |
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Q39359080 | Expanding Axonal Transcriptome Brings New Functions for Axonally Synthesized Proteins in Health and Disease |
Q90408362 | Extensive splicing changes in an ALS/FTD transgenic mouse model overexpressing cytoplasmic fused in sarcoma |
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Q30848999 | Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis |
Q91741049 | Omics Approach to Axonal Dysfunction of Motor Neurons in Amyotrophic Lateral Sclerosis (ALS) |
Q64228212 | Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis |
Q90024987 | Protein functional annotation of simultaneously improved stability, accuracy and false discovery rate achieved by a sequence-based deep learning |
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Q54125538 | Synaptic Paths to Neurodegeneration: The Emerging Role of TDP-43 and FUS in Synaptic Functions. |
Q47427810 | To the end of the line: Axonal mRNA transport and local translation in health and neurodegenerative disease. |
Q61807545 | Transgenic and physiological mouse models give insights into different aspects of amyotrophic lateral sclerosis |
Q102152356 | Ubiquitin signaling in neurodegenerative diseases: an autophagy and proteasome perspective |
Q58700147 | genetically interacts with the ALS-associated orthologue and mediates its toxicity |
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