Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway

Nucleocytoplasmic Proteomic Analysis Uncovers eRF1 and Nonsense-Mediated Decay as Modifiers of ALS/FTD C9orf72 Toxicity

(2020) Juan A. Ortega et al.   NEURON

G4C2 Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD

(2020) Alyssa N. Coyne et al.   NEURON

ALS-linked FUS mutations confer loss and gain of function in the nucleus by promoting excessive formation of dysfunctional paraspeckles

(2019) Haiyan An et al.   Acta Neuropathologica Communications

RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43

(2019) Jacob R. Mann et al.   NEURON

Quantitative proteomics identifies proteins that resist translational repression and become dysregulated in ALS-FUS

(2019) Desiree M Baron et al.   HUMAN MOLECULAR GENETICS

The RNA-binding protein FUS/TLS undergoes calcium-mediated nuclear egress during excitotoxic stress and is required for GRIA2 mRNA processing

(2019) Maeve Tischbein et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Improving homology-directed repair efficiency in human stem cells

(2019) William C. Skarnes et al.   METHODS

Nucleocytoplasmic transport defects in neurodegeneration — Cause or consequence?

(2019) Saskia Hutten et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Physiological, Pathological, and Targetable Membraneless Organelles in Neurons

(2019) Veronica H. Ryan et al.   TRENDS IN NEUROSCIENCES

Modulation of actin polymerization affects nucleocytoplasmic transport in multiple forms of amyotrophic lateral sclerosis

(2019) Anthony Giampetruzzi et al.   Nature Communications

C9orf72-generated poly-GR and poly-PR do not directly interfere with nucleocytoplasmic transport

(2019) Joni Vanneste et al.   Scientific Reports

Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization

(2019) Ian Casci et al.   Nature Communications

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

(2018) Ching-Chieh Chou et al.   NATURE NEUROSCIENCE

EasyFRAP-web: a web-based tool for the analysis of fluorescence recovery after photobleaching data

(2018) Grigorios Koulouras et al.   NUCLEIC ACIDS RESEARCH

RNA buffers the phase separation behavior of prion-like RNA binding proteins

(2018) Shovamayee Maharana et al.   SCIENCE

TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia

(2018) Hilary C. Archbold et al.   Scientific Reports

Intrinsically Disordered Regions Can Contribute Promiscuous Interactions to RNP Granule Assembly

(2018) David S.W. Protter et al.   Cell Reports

Tau Protein Disrupts Nucleocytoplasmic Transport in Alzheimer’s Disease

(2018) Bahareh Eftekharzadeh et al.   NEURON

Mutant FUS causes DNA ligation defects to inhibit oxidative damage repair in Amyotrophic Lateral Sclerosis

(2018) Haibo Wang et al.   Nature Communications

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

(2018) Marisa Kamelgarn et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Calpain-Dependent Degradation of Nucleoporins Contributes to Motor Neuron Death in a Mouse Model of Chronic Excitotoxicity

(2017) Kaori Sugiyama et al.   JOURNAL OF NEUROSCIENCE

Toxic PRnpoly-dipeptides encoded by theC9orf72repeat expansion block nuclear import and export

(2017) Kevin Y. Shi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2017) Reddy Ranjith K. Sama et al.   Scientific Reports

Calpain-dependent disruption of nucleo-cytoplasmic transport in ALS motor neurons

(2017) Takenari Yamashita et al.   Scientific Reports

Proteomic analysis of FUS interacting proteins provides insights into FUS function and its role in ALS

(2016) Marisa Kamelgarn et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Lamin Dysfunction Mediates Neurodegeneration in Tauopathies

(2016) Bess Frost et al.   CURRENT BIOLOGY

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

(2016) Radu Stoica et al.   EMBO REPORTS

Human SOD1 ALS Mutations in a Drosophila Knock-In Model Cause Severe Phenotypes and Reveal Dosage-Sensitive Gain- and Loss-of-Function Components

(2016) Aslı Şahin et al.   GENETICS

The Ultrastructural Signature of Human Embryonic Stem Cells

(2016) Jean M. Underwood et al.   JOURNAL OF CELLULAR BIOCHEMISTRY

Transport Selectivity of Nuclear Pores, Phase Separation, and Membraneless Organelles

(2016) H. Broder Schmidt et al.   TRENDS IN BIOCHEMICAL SCIENCES

Human SOD1 ALS Mutations in a Drosophila Knock-In Model Cause Severe Phenotypes and Reveal Dosage-Sensitive Gain- and Loss-of-Function Components

(2016) Aslı Şahin et al.   GENETICS

Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD

(2016) Steven Boeynaems et al.   Scientific Reports

Genome-wide RNA-Seq of Human Motor Neurons Implicates Selective ER Stress Activation in Spinal Muscular Atrophy

(2015) Shi-Yan Ng et al.   Cell Stem Cell

Nuclear pore proteins and the control of genome functions

(2015) Arkaitz Ibarra et al.   GENES & DEVELOPMENT

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

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

(2015) Brian D. Freibaum et al.   NATURE

Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS

(2015) Ana Jovičić et al.   NATURE NEUROSCIENCE

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

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA

(2015) A. C. Woerner et al.   SCIENCE

Functions of FUS/TLS From DNA Repair to Stress Response: Implications for ALS

(2014) Reddy Ranjith Kumar Sama et al.   ASN Neuro

Identification of Long-Lived Proteins Reveals Exceptional Stability of Essential Cellular Structures

(2013) Brandon H. Toyama et al.   CELL

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

(2013) Desiree M Baron et al.   Molecular Neurodegeneration

A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43

(2011) N. A. Lanson et al.   HUMAN MOLECULAR GENETICS

Expression of human FUS protein in Drosophila leads to progressive neurodegeneration

(2011) Yanbo Chen et al.   Protein & Cell

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

Fusgene mutations in familial and sporadic amyotrophic lateral sclerosis

(2010) Rosa Rademakers et al.   MUSCLE & NERVE

Age-Dependent Deterioration of Nuclear Pore Complexes Causes a Loss of Nuclear Integrity in Postmitotic Cells

(2009) Maximiliano A. D'Angelo et al.   CELL

Analyzing real-time PCR data by the comparative CT method

(2008) Thomas D Schmittgen et al.   Nature Protocols