Widespread Mislocalization of FUS Is Associated With Mitochondrial Abnormalities in Skeletal Muscle in Amyotrophic Lateral Sclerosis With FUS Mutations

Identification of a novel interaction of FUS and syntaphilin may explain synaptic and mitochondrial abnormalities caused by ALS mutations

(2021) Shaakir Salam et al.   Scientific Reports

Expansion of GGC Repeat in GIPC1 Is Associated with Oculopharyngodistal Myopathy

(2020) Jianwen Deng et al.   AMERICAN JOURNAL OF HUMAN GENETICS

ROS induced p53 activation: DNA damage, redox signaling or both?

(2020) Tao Shi et al.   ANTIOXIDANTS & REDOX SIGNALING

ALS/FTD-associated protein FUS induces mitochondrial dysfunction by preferentially sequestering respiratory chain complex mRNAs

(2020) Yueh-Lin Tsai et al.   GENES & DEVELOPMENT

FUS P525L mutation causing amyotrophic lateral sclerosis and movement disorders

(2020) Binbin Zhou et al.   Brain and Behavior

RNA-Binding Proteins Implicated in Mitochondrial Damage and Mitophagy

(2020) Stylianos Ravanidis et al.   Frontiers in Cell and Developmental Biology

Widespread FUS mislocalization is a molecular hallmark of amyotrophic lateral sclerosis

(2019) Giulia E Tyzack et al.   BRAIN

FUS-mediated regulation of acetylcholine receptor transcription at neuromuscular junctions is compromised in amyotrophic lateral sclerosis

(2019) Gina Picchiarelli et al.   NATURE NEUROSCIENCE

Stress granules and neurodegeneration

(2019) Benjamin Wolozin et al.   NATURE REVIEWS NEUROSCIENCE

Differentiation but not ALS mutations in FUS rewires motor neuron metabolism

(2019) Tijs Vandoorne et al.   Nature Communications

Phosphorylated TDP-43 aggregates in skeletal and cardiac muscle are a marker of myogenic degeneration in amyotrophic lateral sclerosis and various conditions

(2019) Fumiaki Mori et al.   Acta Neuropathologica Communications

Novel and Recurrent Mutations in a Cohort of Chinese Patients With Young-Onset Amyotrophic Lateral Sclerosis

(2019) Jianwen Deng et al.   Frontiers in Neuroscience

ALS Genes in the Genomic Era and their Implications for FTD

(2018) Hung Phuoc Nguyen et al.   TRENDS IN GENETICS

TDP-43 induces p53-mediated cell death of cortical progenitors and immature neurons

(2018) Miriam A. Vogt et al.   Scientific Reports

Phosphorylated TDP-43 (pTDP-43) aggregates in the axial skeletal muscle of patients with sporadic and familial amyotrophic lateral sclerosis

(2018) Matthew D. Cykowski et al.   Acta Neuropathologica Communications

FUS interacts with ATP synthase beta subunit and induces mitochondrial unfolded protein response in cellular and animal models

(2018) Jianwen Deng et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis

(2016) Jean-Philippe Loeffler et al.   BRAIN PATHOLOGY

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

(2016) Radu Stoica et al.   EMBO REPORTS

Mutation spectrum of Chinese patients with familial and sporadic amyotrophic lateral sclerosis

(2016) Qing Liu et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

A metabolic switch toward lipid use in glycolytic muscle is an early pathologic event in a mouse model of amyotrophic lateral sclerosis

(2015) L. Palamiuc et al.   EMBO Molecular Medicine

Clinical and Muscle Imaging Findings in 14 Mainland Chinese Patients with Oculopharyngodistal Myopathy

(2015) Juan Zhao et al.   PLoS One

Activation of the endoplasmic reticulum stress response in skeletal muscle of G93A*SOD1 amyotrophic lateral sclerosis mice

(2015) Dapeng Chen et al.   Frontiers in Cellular Neuroscience

FUS Interacts with HSP60 to Promote Mitochondrial Damage

(2015) Jianwen Deng et al.   PLoS Genetics

ALS-FUS pathology revisited: singleton FUS mutations and an unusual case with both a FUS and TARDBP mutation

(2015) Andrew King et al.   Acta Neuropathologica Communications

Neuronal cells but not muscle cells are resistant to oxidative stress mediated protein misfolding and cell death: Role of molecular chaperones

(2014) Arunabh Bhattacharya et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Mitochondrial abnormalities and low grade inflammation are present in the skeletal muscle of a minority of patients with amyotrophic lateral sclerosis; an observational myopathology study

(2014) Safa Al-Sarraj et al.   Acta Neuropathologica Communications

TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis

(2014) Matthew D Cykowski et al.   Acta Neuropathologica Communications

Mutations in the 3′ untranslated region of FUS causing FUS overexpression are associated with amyotrophic lateral sclerosis

(2013) Mario Sabatelli et al.   HUMAN MOLECULAR GENETICS

The genetics and neuropathology of amyotrophic lateral sclerosis

(2012) Ammar Al-Chalabi et al.   ACTA NEUROPATHOLOGICA

Detecting differential usage of exons from RNA-seq data

(2012) S. Anders et al.   GENOME RESEARCH

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

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

DNA-fragmentation and apoptosis-related proteins of muscle cells in motor neuron disorders

(2010) D. S. Tews et al.   ACTA NEUROLOGICA SCANDINAVICA

Association of paraspinal and diaphragm denervation in ALS

(2010) Mamede de Carvalho et al.   Amyotrophic Lateral Sclerosis

Mitochondrial Respiratory Chain Dysfunction in Muscle From Patients With Amyotrophic Lateral Sclerosis

(2010) Veronica Crugnola et al.   ARCHIVES OF NEUROLOGY

Amyotrophic Lateral Sclerosis–Plus Syndrome With TAR DNA-Binding Protein-43 Pathology

(2009) Leo F. McCluskey et al.   ARCHIVES OF NEUROLOGY

A new subtype of frontotemporal lobar degeneration with FUS pathology

(2009) M. Neumann et al.   BRAIN

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

(2009) C. Vance et al.   SCIENCE