FUS(1-359) transgenic mice as a model of ALS: pathophysiological and molecular aspects of the proteinopathy

Neuron-specific alternative splicing of transcriptional machineries: Implications for neurodevelopmental disorders

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Intron retention and nuclear loss of SFPQ are molecular hallmarks of ALS

(2018) Raphaelle Luisier et al.   Nature Communications

TDP-43 and FUS en route from the nucleus to the cytoplasm

(2017) Helena Ederle et al.   FEBS LETTERS

Mouse models of frontotemporal dementia: A comparison of phenotypes with clinical symptomatology

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Microglia Responses in Acute and Chronic Neurological Diseases: What Microglia-Specific Transcriptomic Studies Taught (and did Not Teach) Us

(2017) Hélène E. Hirbec et al.   Frontiers in Aging Neuroscience

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

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

Retrotransposon activation contributes to neurodegeneration in a Drosophila TDP-43 model of ALS

(2017) Lisa Krug et al.   PLoS Genetics

Microbiota, Immune Subversion, and Chronic Inflammation

(2017) Carolyn D. Kramer et al.   Frontiers in Immunology

Amyotrophic lateral sclerosis

(2017) Orla Hardiman et al.   Nature Reviews Disease Primers

Dendritic Homeostasis Disruption in a Novel Frontotemporal Dementia Mouse Model Expressing Cytoplasmic Fused in Sarcoma

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Protein misfolding in neurodegenerative diseases: implications and strategies

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Differential expression of tetraspanin superfamily members in dendritic cell subsets

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

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Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis

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Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss

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IL-23 Inhibits Melanoma Development by Augmenting DNA Repair and Modulating T Cell Subpopulations

(2016) Tahseen H. Nasti et al.   JOURNAL OF IMMUNOLOGY

IL-10 Controls Early Microglial Phenotypes and Disease Onset in ALS Caused by Misfolded Superoxide Dismutase 1

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Activation of HIPK2 Promotes ER Stress-Mediated Neurodegeneration in Amyotrophic Lateral Sclerosis

(2016) Sebum Lee et al.   NEURON

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

(2016) Aarti Sharma et al.   Nature Communications

Zbtb20 promotes astrocytogenesis during neocortical development

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Loss of ZBTB20 impairs circadian output and leads to unimodal behavioral rhythms

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Microglial Activation as a Compelling Target for Treating Acute Traumatic Brain Injury

(2015) Chung-Ching Chio et al.   CURRENT MEDICINAL CHEMISTRY

Emerging mechanisms of molecular pathology in ALS

(2015) Owen M. Peters et al.   JOURNAL OF CLINICAL INVESTIGATION

PPLine: An Automated Pipeline for SNP, SAP, and Splice Variant Detection in the Context of Proteogenomics

(2015) George Sergeevich Krasnov et al.   JOURNAL OF PROTEOME RESEARCH

Brca1 is expressed in human microglia and is dysregulated in human and animal model of ALS

(2015) Harun Najib Noristani et al.   Molecular Neurodegeneration

Microexons on the brain

(2015) Brooke LaFlamme   NATURE GENETICS

Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions

(2015) Natalie E. Farrawell et al.   Scientific Reports

Meningeal Infiltration of the Spinal Cord by Non-Classically Activated B Cells is Associated with Chronic Disease Course in a Spontaneous B Cell-Dependent Model of CNS Autoimmune Disease

(2015) Amy K. Dang et al.   Frontiers in Immunology

Transcriptomic Changes Due to Cytoplasmic TDP-43 Expression Reveal Dysregulation of Histone Transcripts and Nuclear Chromatin

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A Highly Conserved Program of Neuronal Microexons Is Misregulated in Autistic Brains

(2014) Manuel Irimia et al.   CELL

The role of PI3K/AKT/mTOR pathway in the modulation of autophagy and the clearance of protein aggregates in neurodegeneration

(2014) David Heras-Sandoval et al.   CELLULAR SIGNALLING

B-cell receptor signalling and its crosstalk with other pathways in normal and malignant cells

(2014) Vaclav Seda et al.   EUROPEAN JOURNAL OF HAEMATOLOGY

Lymphocytes and autoimmunity after spinal cord injury

(2014) T. Bucky Jones   EXPERIMENTAL NEUROLOGY

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(2014) B. Linnartz-Gerlach et al.   GLYCOBIOLOGY

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

Cerebellar degeneration-related autoantigen 1 (CDR1) gene expression in Alzheimer’s disease

(2014) Paolo Bosco et al.   NEUROLOGICAL SCIENCES

Blood-spinal cord barrier disruption contributes to early motor-neuron degeneration in ALS-model mice

(2014) E. A. Winkler et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A plural role for lipids in motor neuron diseases: energy, signaling and structure

(2014) Florent Schmitt et al.   Frontiers in Cellular Neuroscience

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(2013) Philip McGoldrick et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Inference on periodicity of circadian time series

(2013) M. J. Costa et al.   BIOSTATISTICS

Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasm

(2013) Tatyana A Shelkovnikova et al.   CELL CYCLE

LINGO-1 Receptor Promotes Neuronal Apoptosis by Inhibiting WNK3 Kinase Activity

(2013) Zhaohuan Zhang 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

Genetics of amyotrophic lateral sclerosis: an update

(2013) Sheng Chen et al.   Molecular Neurodegeneration

Widespread splicing changes in human brain development and aging

(2013) P. Mazin et al.   Molecular Systems Biology

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(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

RNA-Seq Profiling of Spinal Cord Motor Neurons from a Presymptomatic SOD1 ALS Mouse

(2013) Urmi Bandyopadhyay et al.   PLoS One

Intricate interplay between astrocytes and motor neurons in ALS

(2013) H. P. Phatnani et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Metabolic signatures of amyotrophic lateral sclerosis reveal insights into disease pathogenesis

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

A Neurodegeneration-Specific Gene-Expression Signature of Acutely Isolated Microglia from an Amyotrophic Lateral Sclerosis Mouse Model

(2013) Isaac M. Chiu et al.   Cell Reports

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ATRX induction by mutant huntingtin via Cdx2 modulates heterochromatin condensation and pathology in Huntington's disease

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Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS

(2012) Bing Liao et al.   EXPERIMENTAL NEUROLOGY

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

Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicity

(2012) S. J. Weisberg et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43

(2011) Magdalini Polymenidou et al.   NATURE NEUROSCIENCE

Decreased expression of Bmal2 in patients with Parkinson's disease

(2011) Hui Ding et al.   NEUROSCIENCE LETTERS

FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration

(2011) Cao Huang et al.   PLoS Genetics

Expression of human FUS protein in Drosophila leads to progressive neurodegeneration

(2011) Yanbo Chen et al.   Protein & Cell

Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis

(2010) Brian C. Kraemer et al.   ACTA NEUROPATHOLOGICA

FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis

(2010) Han-Xiang Deng et al.   ANNALS OF NEUROLOGY

Dual Induction of TREM2 and Tolerance-Related Transcript, Tmem176b, in Amyloid Transgenic Mice: Implications for Vaccine-Based Therapies for Alzheimer's Disease

(2010) Benoit Melchior et al.   ASN Neuro

The Role of CD80/CD86 in Generation and Maintenance of Functional Virus-Specific CD8+ T Cells in Mice Infected with Lymphocytic Choriomeningitis Virus

(2010) M. Grujic et al.   JOURNAL OF IMMUNOLOGY

Monitoring systemic oxidative stress in an animal model of amyotrophic lateral sclerosis

(2010) Francisco Javier Miana-Mena et al.   JOURNAL OF NEUROLOGY

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

Gene ontology analysis for RNA-seq: accounting for selection bias

(2010) Matthew D Young et al.   GENOME BIOLOGY

Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease

(2009) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

Downregulation of uridine-cytidine kinase like-1 decreases proliferation and enhances tumor susceptibility to lysis by apoptotic agents and natural killer cells

(2009) Elise C. Ambrose et al.   APOPTOSIS

edgeR: a Bioconductor package for differential expression analysis of digital gene expression data

(2009) M. D. Robinson et al.   BIOINFORMATICS

A new subtype of frontotemporal lobar degeneration with FUS pathology

(2009) M. Neumann et al.   BRAIN

CD22 and Siglec-G: B-cell inhibitory receptors with distinct functions

(2009) Lars Nitschke   IMMUNOLOGICAL REVIEWS

Systematic and integrative analysis of large gene lists using DAVID bioinformatics resources

(2009) Da Wei Huang et al.   Nature Protocols

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

Upregulation of GSK3β expression in frontal and temporal cortex in ALS with cognitive impairment (ALSci)

(2008) Wencheng Yang et al.   BRAIN RESEARCH

IL-6 and metabolism—new evidence and new questions

(2008) A. Krook   DIABETOLOGIA

Misfolded proteins partition between two distinct quality control compartments

(2008) Daniel Kaganovich et al.   NATURE

Developmental Regulation of TREM2 and DAP12 Expression in the Murine CNS: Implications for Nasu-Hakola Disease

(2008) J. Cameron Thrash et al.   NEUROCHEMICAL RESEARCH

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2008) J. Sreedharan et al.   SCIENCE