The FUS protein: Physiological functions and a role in amyotrophic lateral sclerosis

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

ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP

(2015) Shuying Sun et al.   Nature Communications

Highly efficient cell-type-specific gene inactivation reveals a key function for the Drosophila FUS homolog cabeza in neurons

(2015) Marie Frickenhaus et al.   Scientific Reports

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

The ALS gene FUS regulates synaptic transmission at the Drosophila neuromuscular junction

(2014) James B. Machamer et al.   HUMAN MOLECULAR GENETICS

Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms

(2014) Tatyana A. Shelkovnikova et al.   HUMAN MOLECULAR GENETICS

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

FUS is sequestered in nuclear aggregates in ALS patient fibroblasts

(2014) Jacob C. Schwartz et al.   MOLECULAR BIOLOGY OF THE CELL

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

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

Phosphorylation-Regulated Binding of RNA Polymerase II to Fibrous Polymers of Low-Complexity Domains

(2013) Ilmin Kwon et al.   CELL

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-associated mutations in FUS disrupt the axonal distribution and function of SMN

(2013) Ewout J.N. Groen 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

Intranuclear Aggregation of Mutant FUS/TLS as a Molecular Pathomechanism of Amyotrophic Lateral Sclerosis

(2013) Takao Nomura 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

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

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

FUS/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress

(2013) Reddy Ranjith K. Sama et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Defects in Synapse Structure and Function Precede Motor Neuron Degeneration in Drosophila Models of FUS-Related ALS

(2013) M. Shahidullah et al.   JOURNAL OF NEUROSCIENCE

Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons

(2013) Wen-Yuan Wang et al.   NATURE NEUROSCIENCE

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

The epidemiology of ALS: a conspiracy of genes, environment and time

(2013) Ammar Al-Chalabi et al.   Nature Reviews Neurology

PARP-1 dependent recruitment of the amyotrophic lateral sclerosis-associated protein FUS/TLS to sites of oxidative DNA damage

(2013) Stuart L. Rulten et al.   NUCLEIC ACIDS RESEARCH

FUS regulates genes coding for RNA-binding proteins in neurons by binding to their highly conserved introns

(2013) T. Nakaya et al.   RNA

FUS-regulated region- and cell-type-specific transcriptome is associated with cell selectivity in ALS/FTLD

(2013) Yusuke Fujioka et al.   Scientific Reports

RNA Seeds Higher-Order Assembly of FUS Protein

(2013) Jacob C. Schwartz et al.   Cell Reports

The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis

(2013) Yoshinori Nishimoto et al.   Molecular Brain

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

(2013) Yueqin Zhou et al.   PLoS Genetics

Localization of fused in sarcoma (FUS) protein to the post-synaptic density in the brain

(2012) Naoya Aoki 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

Cell-free Formation of RNA Granules: Low Complexity Sequence Domains Form Dynamic Fibers within Hydrogels

(2012) Masato Kato et al.   CELL

Cell-free Formation of RNA Granules: Bound RNAs Identify Features and Components of Cellular Assemblies

(2012) Tina W. Han et al.   CELL

Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS

(2012) Dorothee Dormann et al.   EMBO JOURNAL

Alternative 3′-end processing of long noncoding RNA initiates construction of nuclear paraspeckles

(2012) Takao Naganuma et al.   EMBO JOURNAL

Loss of fused in sarcoma (FUS) promotes pathological Tau splicing

(2012) Denise Orozco et al.   EMBO REPORTS

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

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

Proteinopathies, neurodegenerative disorders with protein aggregation-based pathology

(2012) T. A. Shelkovnikova et al.   MOLECULAR BIOLOGY

Motor neuron apoptosis and neuromuscular junction perturbation are prominent features in a Drosophila model of Fus-mediated ALS

(2012) Ruohan Xia 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

Knockdown of the Drosophila Fused in Sarcoma (FUS) Homologue Causes Deficient Locomotive Behavior and Shortening of Motoneuron Terminal Branches

(2012) Hiroshi Sasayama et al.   PLoS One

Structural and energetic basis of ALS-causing mutations in the atypical proline–tyrosine nuclear localization signal of the Fused in Sarcoma protein (FUS)

(2012) Zi Chao Zhang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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

FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA

(2012) Tomohiro Yamazaki et al.   Cell Reports

PGC-1α Regulates Translocated in Liposarcoma Activity: Role in Oxidative Stress Gene Expression

(2011) Cristina Sánchez-Ramos et al.   ANTIOXIDANTS & REDOX SIGNALING

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

The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span

(2011) Ji-Wu Wang et al.   JOURNAL OF CLINICAL INVESTIGATION

Accumulation of insoluble forms of FUS protein correlates with toxicity in Drosophila

(2011) Laetitia Miguel et al.   NEUROBIOLOGY OF AGING

A yeast functional screen predicts new candidate ALS disease genes

(2011) J. Couthouis et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Yeast Model of FUS/TLS-Dependent Cytotoxicity

(2011) Shulin Ju et al.   PLOS BIOLOGY

Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS

(2011) Zhihui Sun 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

FUS/TLS forms cytoplasmic aggregates, inhibits cell growth and interacts with TDP-43 in a yeast model of amyotrophic lateral sclerosis

(2011) Dmitry Kryndushkin et al.   Protein & Cell

Expression of human FUS protein in Drosophila leads to progressive neurodegeneration

(2011) Yanbo Chen et al.   Protein & Cell

Expression of human FUS/TLS in yeast leads to protein aggregation and cytotoxicity, recapitulating key features of FUS proteinopathy

(2011) Kazuo Fushimi et al.   Protein & Cell

TEThered to Runx: Novel binding partners for runx factors

(2010) Xiaodong Li et al.   BLOOD CELLS MOLECULES AND DISEASES

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

(2010) Dorothee Dormann et al.   EMBO JOURNAL

De novo truncating FUS gene mutation as a cause of sporadic amyotrophic lateral sclerosis

(2010) Mariely DeJesus-Hernandez et al.   HUMAN MUTATION

Prion-like disorders: blurring the divide between transmissibility and infectivity

(2010) M. Cushman et al.   JOURNAL OF CELL SCIENCE

Pathogenicity of exonic indels in fused in sarcoma in amyotrophic lateral sclerosis

(2010) Nicola J. Rutherford et al.   NEUROBIOLOGY OF AGING

Novel missense and truncating mutations in FUS/TLS in familial ALS

(2010) S. Waibel et al.   NEUROLOGY

Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations

(2010) D. Baumer et al.   NEUROLOGY

A protein-protein interaction of stress-responsive myosin VI endowed to inhibit neural progenitor self-replication with RNA binding protein, TLS, in murine hippocampus

(2009) Takeshi Takarada et al.   JOURNAL OF NEUROCHEMISTRY

TLS Inhibits RNA Polymerase III Transcription

(2009) A. Y. Tan et al.   MOLECULAR AND CELLULAR BIOLOGY

Compositional Determinants of Prion Formation in Yeast

(2009) J. A. Toombs et al.   MOLECULAR AND CELLULAR 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

The TET Family of Proteins: Functions and Roles in Disease

(2009) A. Y. Tan et al.   Journal of Molecular Cell Biology

Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription

(2008) Xiangting Wang et al.   NATURE