ALS mutations of FUS suppress protein translation and disrupt the regulation of nonsense-mediated decay

ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS

(2018) Jone López-Erauskin et al.   NEURON

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

(2017) Steven Boeynaems et al.   MOLECULAR CELL

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

(2017) Ian R. Mackenzie et al.   NEURON

Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons

(2017) Pallavi P. Gopal et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Ribosomopathies: There’s strength in numbers

(2017) Eric W. Mills et al.   SCIENCE

Chronically stressed or stress-preconditioned neurons fail to maintain stress granule assembly

(2017) Tatyana A Shelkovnikova et al.   Cell Death & Disease

The Dual Role of Microglia in ALS: Mechanisms and Therapeutic Approaches

(2017) Maria Concetta Geloso et al.   Frontiers in Aging Neuroscience

The Antagonistic Gene Paralogs Upf3a and Upf3b Govern Nonsense-Mediated RNA Decay

(2016) Eleen Y. Shum et al.   CELL

Mechanism and regulation of the nonsense-mediated decay pathway

(2016) Nele Hug et al.   NUCLEIC ACIDS RESEARCH

Enrichr: a comprehensive gene set enrichment analysis web server 2016 update

(2016) Maxim V. Kuleshov et al.   NUCLEIC ACIDS RESEARCH

Principles and Properties of Stress Granules

(2016) David S.W. Protter et al.   TRENDS IN CELL BIOLOGY

Hyperphosphorylation amplifies UPF1 activity to resolve stalls in nonsense-mediated mRNA decay

(2016) Sébastien Durand et al.   Nature Communications

ATP hydrolysis by UPF1 is required for efficient translation termination at premature stop codons

(2016) Lucas D. Serdar et al.   Nature Communications

Phase Separation by Low Complexity Domains Promotes Stress Granule Assembly and Drives Pathological Fibrillization

(2015) Amandine Molliex et al.   CELL

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

(2015) Avinash Patel et al.   CELL

The function of RNA-binding proteins at the synapse: implications for neurodegeneration

(2015) Chantelle F. Sephton et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Subcellular localization and RNAs determine FUS architecture in different cellular compartments

(2015) Liuqing Yang et al.   HUMAN MOLECULAR GENETICS

DISEASES: Text mining and data integration of disease–gene associations

(2015) Sune Pletscher-Frankild et al.   METHODS

Target Discrimination in Nonsense-Mediated mRNA Decay Requires Upf1 ATPase Activity

(2015) Suzanne R. Lee et al.   MOLECULAR CELL

ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function

(2015) Tetsuro Murakami et al.   NEURON

Translation initiation factor eIF3 promotes programmed stop codon readthrough

(2015) Petra Beznosková et al.   NUCLEIC ACIDS RESEARCH

Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1

(2015) Sami J. Barmada et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Attenuation of nonsense-mediated mRNA decay facilitates the response to chemotherapeutics

(2015) Maximilian W. Popp et al.   Nature Communications

FUS regulates AMPA receptor function and FTLD/ALS-associated behaviour via GluA1 mRNA stabilization

(2015) Tsuyoshi Udagawa et al.   Nature Communications

Preservation of forelimb function by UPF1 gene therapy in a rat model of TDP-43-induced motor paralysis

(2014) K L Jackson et al.   GENE THERAPY

Self-assembled FUS binds active chromatin and regulates gene transcription

(2014) Liuqing Yang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Regulation of nonsense-mediated mRNA decay: Implications for physiology and disease

(2013) Rachid Karam et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

Enrichr: interactive and collaborative HTML5 gene list enrichment analysis tool

(2013) Edward Y Chen et al.   BMC BIOINFORMATICS

Role of SMG-1-mediated Upf1 phosphorylation in mammalian nonsense-mediated mRNA decay

(2013) Akio Yamashita   GENES TO CELLS

The UPF3B gene, implicated in intellectual disability, autism, ADHD and childhood onset schizophrenia regulates neural progenitor cell behaviour and neuronal outgrowth

(2013) Lachlan A. Jolly 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

HDAC6 Regulates Mutant SOD1 Aggregation through Two SMIR Motifs and Tubulin Acetylation

(2013) Jozsef Gal et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The RNA-binding protein Fus directs translation of localized mRNAs in APC-RNP granules

(2013) Kyota Yasuda et al.   JOURNAL OF CELL BIOLOGY

Amyotrophic lateral sclerosis-linked FUS/TLS alters stress granule assembly and dynamics

(2013) Desiree M Baron et al.   Molecular Neurodegeneration

The Central Dogma Decentralized: New Perspectives on RNA Function and Local Translation in Neurons

(2013) Christine E. Holt et al.   NEURON

ALS-Associated FUS Mutations Result in Compromised FUS Alternative Splicing and Autoregulation

(2013) Yueqin Zhou et al.   PLoS Genetics

FUS binds the CTD of RNA polymerase II and regulates its phosphorylation at Ser2

(2012) J. C. Schwartz et al.   GENES & DEVELOPMENT

Axonal mRNA localization and local protein synthesis in nervous system assembly, maintenance and repair

(2012) Hosung Jung et al.   NATURE REVIEWS NEUROSCIENCE

Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions

(2012) Shinsuke Ishigaki et al.   Scientific Reports

Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain

(2012) Boris Rogelj et al.   Scientific Reports

Local RNA Translation at the Synapse and in Disease

(2011) L. Liu-Yesucevitz et al.   JOURNAL OF NEUROSCIENCE

RNA Homeostasis Governed by Cell Type-Specific and Branched Feedback Loops Acting on NMD

(2011) Lulu Huang et al.   MOLECULAR CELL

N- and C-terminal Upf1 phosphorylations create binding platforms for SMG-6 and SMG-5:SMG-7 during NMD

(2011) Yukiko Okada-Katsuhata et al.   NUCLEIC ACIDS RESEARCH

Autoregulation of the nonsense-mediated mRNA decay pathway in human cells

(2011) H. Yepiskoposyan et al.   RNA

A Yeast Model of FUS/TLS-Dependent Cytotoxicity

(2011) Shulin Ju et al.   PLOS BIOLOGY

Upf1 ATPase-Dependent mRNP Disassembly Is Required for Completion of Nonsense- Mediated mRNA Decay

(2010) Tobias M. Franks et al.   CELL

Inhibition of eukaryotic translation elongation by cycloheximide and lactimidomycin

(2010) Tilman Schneider-Poetsch et al.   Nature Chemical Biology

The mechanism of eukaryotic translation initiation and principles of its regulation

(2010) Richard J. Jackson et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Nuclear localization sequence of FUS and induction of stress granules by ALS mutants

(2010) Jozsef Gal et al.   NEUROBIOLOGY OF AGING

Efficient protection and isolation of ubiquitylated proteins using tandem ubiquitin-binding entities

(2009) Roland Hjerpe et al.   EMBO REPORTS

SUnSET, a nonradioactive method to monitor protein synthesis

(2009) Enrico K Schmidt et al.   NATURE METHODS

A UPF3-mediated regulatory switch that maintains RNA surveillance

(2009) Wai-Kin Chan et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

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

Rapamycin differentially inhibits S6Ks and 4E-BP1 to mediate cell-type-specific repression of mRNA translation

(2008) A. Y. Choo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA