FUS(1-359) transgenic mice as a model of ALS: pathophysiological and molecular aspects of the proteinopathy

scientific article published on 07 July 2018

FUS(1-359) transgenic mice as a model of ALS: pathophysiological and molecular aspects of the proteinopathy is …
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scholarly articleQ13442814

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P356DOI10.1007/S10048-018-0553-9
P698PubMed publication ID29982879

P50authorPavel MazinQ58335646
Alexey MorozovQ61103806
Mikhail YevgenyevQ88673480
Aleksey UstyugovQ38545717
P2093author name stringAndrey V Maltsev
Ekaterina A Vikhareva
Alexander P Rezvykh
Maria M Chicheva
Sergei Y Funikov
P2860cites workMislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in miceQ48645659
Effect of apigenin, kaempferol and resveratrol on the gene expression and protein secretion of tumor necrosis factor alpha (TNF-α) and interleukin-10 (IL-10) in RAW-264.7 macrophages.Q50521335
Cerebellar degeneration-related autoantigen 1 (CDR1) gene expression in Alzheimer's disease.Q50646415
CD84 mediates CLL-microenvironment interactions.Q51620105
Rodent models of amyotrophic lateral sclerosis.Q51822707
Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death.Q52537887
Dyggve-Melchior-Clausen syndrome and Smith-McCort dysplasia: clinical and molecular findings in three families supporting genetic heterogeneity in Smith-McCort dysplasia.Q52934754
Sensitized RNAi screen of human kinases and phosphatases identifies new regulators of apoptosis and chemoresistance.Q53358479
Intron retention and nuclear loss of SFPQ are molecular hallmarks of ALS.Q55356425
Amyotrophic lateral sclerosisQ56531747
Activation-dependent modulation of B lymphocyte migration to chemokinesQ74247915
Upregulation of GSK3beta expression in frontal and temporal cortex in ALS with cognitive impairment (ALSci)Q80552720
Oxidative and endoplasmic reticulum stress interplay in sporadic amyotrophic lateral sclerosisQ80838012
Monitoring systemic oxidative stress in an animal model of amyotrophic lateral sclerosisQ82607328
Decreased expression of Bmal2 in patients with Parkinson's diseaseQ84318196
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementiaQ24600027
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion diseaseQ24628685
Exome sequencing reveals VCP mutations as a cause of familial ALSQ24631513
A new subtype of frontotemporal lobar degeneration with FUS pathologyQ24647697
Misfolded proteins partition between two distinct quality control compartmentsQ24651093
Mechanisms of FUS mutations in familial amyotrophic lateral sclerosisQ26749169
A comprehensive review of amyotrophic lateral sclerosisQ26775401
Amyotrophic Lateral Sclerosis: New Perpectives and UpdateQ26781051
State of play in amyotrophic lateral sclerosis geneticsQ26864816
RNA-Seq profiling of spinal cord motor neurons from a presymptomatic SOD1 ALS mouseQ27321207
FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degenerationQ27342067
Systematic and integrative analysis of large gene lists using DAVID bioinformatics resourcesQ27860739
edgeR: a Bioconductor package for differential expression analysis of digital gene expression dataQ27860819
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6Q28236805
B-cell receptor signalling and its crosstalk with other pathways in normal and malignant cellsQ28245018
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosisQ28270779
The role of CD80/CD86 in generation and maintenance of functional virus-specific CD8+ T cells in mice infected with lymphocytic choriomeningitis virusQ28286961
SLP76 and SLP65: complex regulation of signalling in lymphocytes and beyondQ28298645
Trimmomatic: a flexible trimmer for Illumina sequence dataQ29547278
HTSeq--a Python framework to work with high-throughput sequencing dataQ29614489
Mutations of optineurin in amyotrophic lateral sclerosisQ29614836
STAR: ultrafast universal RNA-seq alignerQ29615052
Pathogenic superoxide dismutase structure, folding, aggregation and turnoverQ30159887
Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesisQ30494776
Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasmQ30559680
Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusionsQ30661910
Toxicity from different SOD1 mutants dysregulates the complement system and the neuronal regenerative response in ALS motor neuronsQ33282966
Microarray analysis of the cellular pathways involved in the adaptation to and progression of motor neuron injury in the SOD1 G93A mouse model of familial ALS.Q33294971
Modelling amyotrophic lateral sclerosis: progress and possibilitiesQ33746289
Gene ontology analysis for RNA-seq: accounting for selection biasQ33861598
Cloning of mouse BMAL2 and its daily expression profile in the suprachiasmatic nucleus: a remarkable acceleration of Bmal2 sequence divergence after Bmal gene duplicationQ33934943
Dual induction of TREM2 and tolerance-related transcript, Tmem176b, in amyloid transgenic mice: implications for vaccine-based therapies for Alzheimer's diseaseQ33999537
Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS.Q34019767
FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosisQ34118590
Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms.Q34155410
Siglec-mediated regulation of immune cell function in disease.Q34314957
LINGO-1 receptor promotes neuronal apoptosis by inhibiting WNK3 kinase activityQ34332453
Male sterility and enhanced radiation sensitivity in TLS(-/-) miceQ34662602
A neurodegeneration-specific gene-expression signature of acutely isolated microglia from an amyotrophic lateral sclerosis mouse modelQ34746889
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43.Q34982074
ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanismQ35605176
PPLine: An Automated Pipeline for SNP, SAP, and Splice Variant Detection in the Context of ProteogenomicsQ35683877
Transcriptomic Changes Due to Cytoplasmic TDP-43 Expression Reveal Dysregulation of Histone Transcripts and Nuclear ChromatinQ35824582
Motor neuron apoptosis and neuromuscular junction perturbation are prominent features in a Drosophila model of Fus-mediated ALSQ35887683
Brca1 is expressed in human microglia and is dysregulated in human and animal model of ALS.Q35906462
ATRX induction by mutant huntingtin via Cdx2 modulates heterochromatin condensation and pathology in Huntington's diseaseQ36028454
Meningeal Infiltration of the Spinal Cord by Non-Classically Activated B Cells is Associated with Chronic Disease Course in a Spontaneous B Cell-Dependent Model of CNS Autoimmune DiseaseQ36097332
Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicityQ36300695
Retrotransposon activation contributes to neurodegeneration in a Drosophila TDP-43 model of ALSQ36310409
TDP-43 and FUS en route from the nucleus to the cytoplasmQ36336278
Transgenic mouse models of amyotrophic lateral sclerosisQ36468459
ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of functionQ36548825
Expression of human FUS protein in Drosophila leads to progressive neurodegenerationQ36584516
Widespread splicing changes in human brain development and agingQ36587200
Histone deacetylase inhibitors: a novel therapeutic approach to Huntington's disease (complex mechanism of neuronal death).Q36611816
Intricate interplay between astrocytes and motor neurons in ALS.Q36637458
Zbtb20 promotes astrocytogenesis during neocortical developmentQ36719166
Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron lossQ36906420
Metabolic signatures of amyotrophic lateral sclerosis reveal insights into disease pathogenesisQ36967649
Activation of HIPK2 Promotes ER Stress-Mediated Neurodegeneration in Amyotrophic Lateral SclerosisQ37079670
Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic miceQ37132099
Genetics of amyotrophic lateral sclerosis: an update.Q37153922
IL-6 and metabolism-new evidence and new questionsQ37155563
Loss of ZBTB20 impairs circadian output and leads to unimodal behavioral rhythmsQ37276766
CD22 and Siglec-G: B-cell inhibitory receptors with distinct functionsQ37548642
A plural role for lipids in motor neuron diseases: energy, signaling and structureQ37594297
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defectsQ37609639
Blood-spinal cord barrier disruption contributes to early motor-neuron degeneration in ALS-model miceQ37659809
Protein misfolding in neurodegenerative diseases: implications and strategiesQ37697832
Siglec functions of microgliaQ38212200
Lymphocytes and autoimmunity after spinal cord injuryQ38228964
The role of PI3K/AKT/mTOR pathway in the modulation of autophagy and the clearance of protein aggregates in neurodegeneration.Q38244879
Microglial activation as a compelling target for treating acute traumatic brain injury.Q38267047
Microglia Responses in Acute and Chronic Neurological Diseases: What Microglia-Specific Transcriptomic Studies Taught (and did Not Teach) Us.Q38430287
Inference on periodicity of circadian time seriesQ38780354
Mouse models of frontotemporal dementia: A comparison of phenotypes with clinical symptomatologyQ39088915
Microbiota, Immune Subversion, and Chronic InflammationQ39203697
A highly conserved program of neuronal microexons is misregulated in autistic brainsQ39403790
IL-10 Controls Early Microglial Phenotypes and Disease Onset in ALS Caused by Misfolded Superoxide Dismutase 1.Q40962491
The Dual Role of Microglia in ALS: Mechanisms and Therapeutic ApproachesQ41107984
Differential expression of tetraspanin superfamily members in dendritic cell subsets.Q41570164
Developmental regulation of TREM2 and DAP12 expression in the murine CNS: implications for Nasu-Hakola diseaseQ41845995
Emerging mechanisms of molecular pathology in ALS.Q42404037
Dendritic Homeostasis Disruption in a Novel Frontotemporal Dementia Mouse Model Expressing Cytoplasmic Fused in SarcomaQ42651961
LINGO-1 and Neurodegeneration: Pathophysiologic Clues for Essential TremorQ43243189
Downregulation of uridine-cytidine kinase like-1 decreases proliferation and enhances tumor susceptibility to lysis by apoptotic agents and natural killer cellsQ43298963
Neuroprotective effects of pyruvate in the quinolinic acid rat model of Huntington's diseaseQ44614374
Regulatory T cells constrain the TCR repertoire of antigen-stimulated conventional CD4 T cellsQ47269379
Neuron-specific alternative splicing of transcriptional machineries: Implications for neurodevelopmental disordersQ47279488
Calcitriol regulates immune genes CD14 and CD180 to modulate LPS responses in human trophoblastsQ47580725
Humanized mutant FUS drives progressive motor neuron degeneration without aggregation in 'FUSDelta14' knockin mice.Q47623937
Neuronal expression of CD22: novel mechanism for inhibiting microglial proinflammatory cytokine productionQ47738457
IL-23 Inhibits Melanoma Development by Augmenting DNA Repair and Modulating T Cell SubpopulationsQ48008318
P433issue3
P407language of work or nameEnglishQ1860
P921main subjectpathophysiologyQ1135939
RNA sequencingQ2542347
AlsQ165383
amyotrophic lateral sclerosisQ206901
P304page(s)189-204
P577publication date2018-07-07
P1433published inNeurogeneticsQ15710048
P1476titleFUS(1-359) transgenic mice as a model of ALS: pathophysiological and molecular aspects of the proteinopathy
P478volume19