Pathogenesis of FUS-associated ALS and FTD: insights from rodent models
Published 2016 View Full Article
- Home
- Publications
- Publication Search
- Publication Details
Title
Pathogenesis of FUS-associated ALS and FTD: insights from rodent models
Authors
Keywords
Amyotrophic lateral sclerosis, Frontotemporal dementia, MND, Frontotemporal lobar degeneration, FUS, FUSopathy, TDP-43
Journal
Acta Neuropathologica Communications
Volume 4, Issue 1, Pages -
Publisher
Springer Nature
Online
2016-09-06
DOI
10.1186/s40478-016-0358-8
References
Ask authors/readers for more resources
Related references
Note: Only part of the references are listed.- Monomethylated and unmethylated FUS exhibit increased binding to Transportin and distinguish FTLD-FUS from ALS-FUS
- (2016) Marc Suárez-Calvet et al. ACTA NEUROPATHOLOGICA
- Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss
- (2016) Jelena Scekic‐Zahirovic et al. EMBO JOURNAL
- ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
- (2016) Aarti Sharma et al. Nature Communications
- De Novo and Rare Variants at Multiple Loci Support the Oligogenic Origins of Atrioventricular Septal Heart Defects
- (2016) James R. Priest et al. PLoS Genetics
- SnapShot: Genetics of ALS and FTD
- (2015) Rita Guerreiro et al. CELL
- ALS mutations in TLS/FUS disrupt target gene expression
- (2015) Tristan H. Coady et al. GENES & DEVELOPMENT
- Phosphorylation of C-terminal tyrosine residue 526 in FUS impairs its nuclear import
- (2015) S. Darovic et al. JOURNAL OF CELL SCIENCE
- Frontotemporal dementia
- (2015) Jee Bang et al. LANCET
- Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice
- (2015) Owen M. Peters et al. NEURON
- C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD
- (2015) Jacqueline G. O’Rourke et al. NEURON
- Oligogenic inheritance of optineurin (OPTN) andC9ORF72mutations in ALS highlights localisation of OPTN in the TDP-43-negative inclusions ofC9ORF72-ALS
- (2015) Joanna J. Bury et al. NEUROPATHOLOGY
- Early lethality and neuronal proteinopathy in mice expressing cytoplasm-targeted FUS that lacks the RNA recognition motif
- (2015) Hannah K. Robinson et al. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
- ALS-FUS pathology revisited: singleton FUS mutations and an unusual case with both a FUS and TARDBP mutation
- (2015) Andrew King et al. Acta Neuropathologica Communications
- FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis
- (2015) Yoshihiro Kino et al. Acta Neuropathologica Communications
- Amyotrophic lateral sclerosis onset is influenced by the burden of rare variants in known amyotrophic lateral sclerosis genes
- (2014) Janet Cady et al. ANNALS OF NEUROLOGY
- ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects
- (2014) Haiyan Qiu et al. JOURNAL OF CLINICAL INVESTIGATION
- FUS is Phosphorylated by DNA-PK and Accumulates in the Cytoplasm after DNA Damage
- (2014) Q. Deng et al. JOURNAL OF NEUROSCIENCE
- Frontotemporal dementia and its subtypes: a genome-wide association study
- (2014) Raffaele Ferrari et al. LANCET NEUROLOGY
- The role of FUS gene variants in neurodegenerative diseases
- (2014) Hao Deng et al. Nature Reviews Neurology
- Activity-dependent FUS dysregulation disrupts synaptic homeostasis
- (2014) C. F. Sephton et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Autosomal dominant inheritance of rapidly progressive amyotrophic lateral sclerosis due to a truncation mutation in the fused in sarcoma (FUS) gene
- (2014) Louisa Kent et al. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
- Diagnosis and Management of Behavioral Variant Frontotemporal Dementia
- (2013) Peter S. Pressman et al. BIOLOGICAL PSYCHIATRY
- Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS
- (2013) Gary A.B. Armstrong et al. HUMAN MOLECULAR GENETICS
- ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules
- (2013) Caroline Vance et al. HUMAN MOLECULAR GENETICS
- The RNA-binding Protein Fused in Sarcoma (FUS) Functions Downstream of Poly(ADP-ribose) Polymerase (PARP) in Response to DNA Damage
- (2013) Adam S. Mastrocola et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Fused in Sarcoma (FUS) Protein Lacking Nuclear Localization Signal (NLS) and Major RNA Binding Motifs Triggers Proteinopathy and Severe Motor Phenotype in Transgenic Mice
- (2013) Tatyana A. Shelkovnikova et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Assessment of cognitive impairment in amyotrophic lateral sclerosis
- (2013) F.J. Rodríguez de Rivera et al. JOURNAL OF THE NEUROLOGICAL SCIENCES
- Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons
- (2013) Wen-Yuan Wang et al. NATURE NEUROSCIENCE
- 25 years of neuroimaging in amyotrophic lateral sclerosis
- (2013) Bradley R. Foerster et al. Nature Reviews Neurology
- The epidemiology of ALS: a conspiracy of genes, environment and time
- (2013) Ammar Al-Chalabi et al. Nature Reviews Neurology
- Deciphering amyotrophic lateral sclerosis: What phenotype, neuropathology and genetics are telling us about pathogenesis
- (2013) John Ravits et al. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
- The genetics and neuropathology of amyotrophic lateral sclerosis
- (2012) Ammar Al-Chalabi et al. ACTA NEUROPATHOLOGICA
- Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion
- (2012) Jacqueline C. Mitchell et al. ACTA NEUROPATHOLOGICA
- The molecular basis of the frontotemporal lobar degeneration–amyotrophic lateral sclerosis spectrum
- (2012) Tim van Langenhove et al. ANNALS OF MEDICINE
- How do C9ORF72 repeat expansions cause amyotrophic lateral sclerosis and frontotemporal dementia
- (2012) Marka van Blitterswijk et al. CURRENT OPINION IN NEUROLOGY
- Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS
- (2012) Dorothee Dormann et al. EMBO JOURNAL
- RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations
- (2012) J. G. Daigle et al. HUMAN MOLECULAR GENETICS
- Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats
- (2012) Cao Huang et al. HUMAN MOLECULAR GENETICS
- Evidence for an oligogenic basis of amyotrophic lateral sclerosis
- (2012) Marka van Blitterswijk et al. HUMAN MOLECULAR GENETICS
- Requirements for Stress Granule Recruitment of Fused in Sarcoma (FUS) and TAR DNA-binding Protein of 43 kDa (TDP-43)
- (2012) Eva Bentmann et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Familial ALS with FUS P525L mutation: two Japanese sisters with multiple systems involvement
- (2012) Yoko Mochizuki et al. JOURNAL OF THE NEUROLOGICAL SCIENCES
- Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis
- (2012) Christophe Verbeeck et al. Molecular Neurodegeneration
- Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs
- (2012) Clotilde Lagier-Tourenne et al. NATURE NEUROSCIENCE
- VAPB and C9orf72 mutations in 1 familial amyotrophic lateral sclerosis patient
- (2012) Marka van Blitterswijk et al. NEUROBIOLOGY OF AGING
- TLS/FUS (translocated in liposarcoma/fused in sarcoma) regulates target gene transcription via single-stranded DNA response elements
- (2012) A. Y. Tan et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutation
- (2011) Ian R. A. Mackenzie et al. ACTA NEUROPATHOLOGICA
- 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
- (2011) Julie S. Snowden et al. ACTA NEUROPATHOLOGICA
- Transportin1: a marker of FTLD-FUS
- (2011) Jack Brelstaff et al. ACTA NEUROPATHOLOGICA
- Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia
- (2011) Katya Rascovsky et al. BRAIN
- ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism
- (2011) Tetsuro Murakami et al. HUMAN MOLECULAR GENETICS
- A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43
- (2011) N. A. Lanson et al. HUMAN MOLECULAR GENETICS
- Arginine methylation by PRMT1 regulates nuclear-cytoplasmic localization and toxicity of FUS/TLS harbouring ALS-linked mutations
- (2011) Miranda L. Tradewell et al. HUMAN MOLECULAR GENETICS
- FUS and TDP43 genetic variability in FTD and CBS
- (2011) Edward D. Huey et al. NEUROBIOLOGY OF AGING
- Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
- (2011) Mariely DeJesus-Hernandez et al. NEURON
- A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
- (2011) Alan E. Renton et al. NEURON
- A Yeast Model of FUS/TLS-Dependent Cytotoxicity
- (2011) Shulin Ju et al. PLOS BIOLOGY
- FUS and TARDBP but Not SOD1 Interact in Genetic Models of Amyotrophic Lateral Sclerosis
- (2011) Edor Kabashi et al. PLoS Genetics
- FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration
- (2011) Cao Huang et al. PLoS Genetics
- Distinct pathological subtypes of FTLD-FUS
- (2010) Ian R. A. Mackenzie et al. ACTA NEUROPATHOLOGICA
- The epidemiology and treatment of ALS: Focus on the heterogeneity of the disease and critical appraisal of therapeutic trials
- (2010) Ettore Beghi et al. Amyotrophic Lateral Sclerosis
- TLS and PRMT1 synergistically coactivate transcription at the survivin promoter through TLS arginine methylation
- (2010) Kun Du et al. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
- Extensive FUS-Immunoreactive Pathology in Juvenile Amyotrophic Lateral Sclerosis with Basophilic Inclusions
- (2010) Eric J. Huang et al. BRAIN PATHOLOGY
- Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules
- (2010) Daryl A. Bosco et al. HUMAN MOLECULAR GENETICS
- TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia
- (2010) Ian RA Mackenzie et al. LANCET NEUROLOGY
- Fusgene mutations in familial and sporadic amyotrophic lateral sclerosis
- (2010) Rosa Rademakers et al. MUSCLE & NERVE
- Genetic contribution of FUS to frontotemporal lobar degeneration
- (2010) T. Van Langenhove et al. NEUROLOGY
- Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations
- (2010) D. Baumer et al. NEUROLOGY
- Oligogenic basis of isolated gonadotropin-releasing hormone deficiency
- (2010) G. P. Sykiotis et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Functional analyses of variants reveal a significant role for dominant negative and common alleles in oligogenic Bardet-Biedl syndrome
- (2010) N. A. Zaghloul et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease
- (2009) Manuela Neumann et al. ACTA NEUROPATHOLOGICA
- Multiple system degeneration with basophilic inclusions in Japanese ALS patients with FUS mutation
- (2009) Takahisa Tateishi et al. ACTA NEUROPATHOLOGICA
- FUS pathology in basophilic inclusion body disease
- (2009) David G. Munoz et al. ACTA NEUROPATHOLOGICA
- Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update
- (2009) Ian R. A. Mackenzie et al. ACTA NEUROPATHOLOGICA
- A new subtype of frontotemporal lobar degeneration with FUS pathology
- (2009) M. Neumann et al. BRAIN
- Mutations of FUS gene in sporadic amyotrophic lateral sclerosis
- (2009) L. Corrado et al. JOURNAL OF MEDICAL GENETICS
- Protein Arginine Methylation in Mammals: Who, What, and Why
- (2009) Mark T. Bedford et al. MOLECULAR CELL
- Analysis of FUS gene mutation in familial amyotrophic lateral sclerosis within an Italian cohort
- (2009) N. Ticozzi et al. NEUROLOGY
- Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
- (2009) C. Vance et al. SCIENCE
- Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis
- (2009) T. J. Kwiatkowski et al. SCIENCE
- TDP-43A315T mutation in familial motor neuron disease
- (2008) Michael A. Gitcho et al. ANNALS OF NEUROLOGY
- The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress response
- (2008) Mattias K Andersson et al. BMC CELL BIOLOGY
- TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
- (2008) J. Sreedharan et al. SCIENCE
Find the ideal target journal for your manuscript
Explore over 38,000 international journals covering a vast array of academic fields.
SearchBecome a Peeref-certified reviewer
The Peeref Institute provides free reviewer training that teaches the core competencies of the academic peer review process.
Get Started