ALS-linked FUS exerts a gain of toxic function involving aberrant p38 MAPK activation

Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice

(2016) Gen Shiihashi et al.   BRAIN

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/FTD‐associated FUS activates GSK‐3β to disrupt the VAPB–PTPIP51 interaction and ER–mitochondria associations

(2016) Radu Stoica et al.   EMBO REPORTS

Axonal transport defects are a common phenotype inDrosophilamodels of ALS

(2016) Katie R. Baldwin et al.   HUMAN MOLECULAR GENETICS

Pathophysiological and diagnostic implications of cortical dysfunction in ALS

(2016) Nimeshan Geevasinga et al.   Nature Reviews Neurology

Extended survival of misfolded G85R SOD1-linked ALS mice by transgenic expression of chaperone Hsp110

(2016) Maria Nagy et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

(2016) Aarti Sharma et al.   Nature Communications

Dysregulated axonal RNA translation in amyotrophic lateral sclerosis

(2016) Kyota Yasuda et al.   Wiley Interdisciplinary Reviews-RNA

Targeting Human Central Nervous System Protein Kinases: An Isoform Selective p38αMAPK Inhibitor That Attenuates Disease Progression in Alzheimer’s Disease Mouse Models

(2015) Saktimayee M. Roy et al.   ACS Chemical Neuroscience

Biochemical Properties and Biological Functions of FET Proteins

(2015) Jacob C. Schwartz et al.   Annual Review of Biochemistry

A fruitful endeavor: Modeling ALS in the fruit fly

(2015) Ian Casci et al.   BRAIN RESEARCH

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

(2015) Avinash Patel et al.   CELL

Analysis of YFP(J16)-R6/2 reporter mice and postmortem brains reveals early pathology and increased vulnerability of callosal axons in Huntington's disease

(2015) Rodolfo G. Gatto et al.   HUMAN MOLECULAR GENETICS

Residue-by-Residue View of In Vitro FUS Granules that Bind the C-Terminal Domain of RNA Polymerase II

(2015) Kathleen A. Burke 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

Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells

(2015) Vincent Picher-Martel et al.   Molecular Brain

An ALS-associated mutation in the FUS 3′-UTR disrupts a microRNA–FUS regulatory circuitry

(2014) Stefano Dini Modigliani et al.   Nature Communications

Intrinsic Membrane Hyperexcitability of Amyotrophic Lateral Sclerosis Patient-Derived Motor Neurons

(2014) Brian J. Wainger et al.   Cell Reports

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

(2013) Ewout J.N. Groen et al.   HUMAN MOLECULAR GENETICS

TARDBPandFUSMutations Associated with Amyotrophic Lateral Sclerosis: Summary and Update

(2013) Serena Lattante et al.   HUMAN MUTATION

Inhibition of Fast Axonal Transport by Pathogenic SOD1 Involves Activation of p38 MAP Kinase

(2013) Gerardo A. Morfini et al.   PLoS One

Molecular chaperone Hsp110 rescues a vesicle transport defect produced by an ALS-associated mutant SOD1 protein in squid axoplasm

(2013) Y. Song et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

MST1 functions as a key modulator of neurodegeneration in a mouse model of ALS

(2013) J. K. Lee et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2013) Yueqin Zhou et al.   PLoS Genetics

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

Alterations in axonal transport motor proteins in sporadic and experimental Parkinson’s disease

(2012) Yaping Chu et al.   BRAIN

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

(2012) Masato Kato et al.   CELL

Truncating mutations inFUS/TLSgive rise to a more aggressive ALS-phenotype than missense mutations: a clinico-genetic study in Germany

(2012) S. Waibel et al.   EUROPEAN JOURNAL OF NEUROLOGY

NIH Image to ImageJ: 25 years of image analysis

(2012) Caroline A Schneider et al.   NATURE METHODS

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

Pathogenic Forms of Tau Inhibit Kinesin-Dependent Axonal Transport through a Mechanism Involving Activation of Axonal Phosphotransferases

(2011) N. M. Kanaan et al.   JOURNAL OF NEUROSCIENCE

The Mammalian Disaggregase Machinery: Hsp110 Synergizes with Hsp70 and Hsp40 to Catalyze Protein Disaggregation and Reactivation in a Cell-Free System

(2011) James Shorter   PLoS One

Proteostasis and Movement Disorders: Parkinson's Disease and Amyotrophic Lateral Sclerosis

(2011) D. A. Bosco et al.   Cold Spring Harbor Perspectives in Biology

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

Axonal Loss and Neurofilament Phosphorylation Changes Accompany Lesion Development and Clinical Progression in Multiple Sclerosis

(2010) Lucas Schirmer et al.   BRAIN PATHOLOGY

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

(2010) Dorothee Dormann et al.   EMBO JOURNAL

Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules

(2010) Daryl A. Bosco et al.   HUMAN MOLECULAR GENETICS

High-efficiency transfection of cultured primary motor neurons to study protein localization, trafficking, and function

(2010) Claudia Fallini et al.   Molecular Neurodegeneration

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

(2010) Daryl A Bosco et al.   NATURE NEUROSCIENCE

Review: Neuromuscular synaptic vulnerability in motor neurone disease: amyotrophic lateral sclerosis and spinal muscular atrophy

(2010) L. M. Murray et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Deficits in axonal transport precede ALS symptoms in vivo

(2010) L. G. Bilsland et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Axonal Transport Defects in Neurodegenerative Diseases

(2009) G. A. Morfini et al.   JOURNAL OF NEUROSCIENCE

Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin

(2009) Gerardo A Morfini et al.   NATURE NEUROSCIENCE

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

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

(2008) Xiangting Wang et al.   NATURE

Plastin 3 Is a Protective Modifier of Autosomal Recessive Spinal Muscular Atrophy

(2008) G. E. Oprea et al.   SCIENCE