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

Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice

(2017) Eva So et al.   HUMAN MOLECULAR GENETICS

Sex specific activation of the ERα axis of the mitochondrial UPR (UPRmt) in the G93A-SOD1 mouse model of familial ALS

(2017) Amanjot K Riar et al.   HUMAN MOLECULAR GENETICS

Dysregulation of the Mitochondrial Unfolded Protein Response Induces Non-Apoptotic Dopaminergic Neurodegeneration inC. elegansModels of Parkinson's Disease

(2017) Bryan A. Martinez et al.   JOURNAL OF NEUROSCIENCE

Activation of the mitochondrial unfolded protein response promotes longevity and dopamine neuron survival in Parkinson’s disease models

(2017) Jason F. Cooper et al.   Scientific Reports

Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis

(2016) Yulei Shang et al.   BRAIN RESEARCH

The Transcription Factor ATF5 Mediates a Mammalian Mitochondrial UPR

(2016) Christopher J. Fiorese 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

Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins

(2016) Antonia Ratti et al.   JOURNAL OF NEUROCHEMISTRY

FUS Interacts with HSP60 to Promote Mitochondrial Damage

(2015) Jianwen Deng et al.   PLoS Genetics

Preparation of Mitochondrial Enriched Fractions for Metabolic Analysis in Drosophila

(2015) Eugenia Villa-Cuesta et al.   Jove-Journal of Visualized Experiments

Mitochondrial Proteostasis in the Control of Aging and Longevity

(2014) Martin Borch Jensen et al.   Cell Metabolism

The good and the bad of being connected: the integrons of aging

(2014) Andrew Dillin et al.   CURRENT OPINION IN CELL BIOLOGY

Loss of LRPPRC causes ATP synthase deficiency

(2014) A. Mourier 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

Caenorhabditis elegans pathways that surveil and defend mitochondria

(2014) Ying Liu et al.   NATURE

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

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

The mitochondrial unfolded protein response, a conserved stress response pathway with implications in health and disease

(2013) V. Jovaisaite et al.   JOURNAL OF EXPERIMENTAL BIOLOGY

SirT3 Regulates the Mitochondrial Unfolded Protein Response

(2013) L. Papa et al.   MOLECULAR AND CELLULAR BIOLOGY

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

(2013) Desiree M Baron et al.   Molecular Neurodegeneration

Mitonuclear protein imbalance as a conserved longevity mechanism

(2013) Riekelt H. Houtkooper et al.   NATURE

Surveillance-Activated Defenses Block the ROS–Induced Mitochondrial Unfolded Protein Response

(2013) Eva D. Runkel et al.   PLoS Genetics

Genetic analysis of mitochondrial protein misfolding in Drosophila melanogaster

(2012) I Pimenta de Castro et al.   CELL DEATH AND DIFFERENTIATION

Requirements for Stress Granule Recruitment of Fused in Sarcoma (FUS) and TAR DNA-binding Protein of 43 kDa (TDP-43)

(2012) Eva Bentmann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structure of the yeast F1Fo-ATP synthase dimer and its role in shaping the mitochondrial cristae

(2012) K. M. Davies et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Cell-Non-Autonomous Nature of Electron Transport Chain-Mediated Longevity

(2011) Jenni Durieux et al.   CELL

Induction of dsRNA-activated protein kinase links mitochondrial unfolded protein response to the pathogenesis of intestinal inflammation

(2011) Eva Rath et al.   GUT

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

Rotation and structure of FoF1-ATP synthase

(2011) D. Okuno et al.   JOURNAL OF BIOCHEMISTRY

Mitochondrial ATP synthase: architecture, function and pathology

(2011) An I. Jonckheere et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Expression of human FUS protein in Drosophila leads to progressive neurodegeneration

(2011) Yanbo Chen et al.   Protein & Cell

Distinct pathological subtypes of FTLD-FUS

(2010) Ian R. A. Mackenzie et al.   ACTA NEUROPATHOLOGICA

Extensive FUS-Immunoreactive Pathology in Juvenile Amyotrophic Lateral Sclerosis with Basophilic Inclusions

(2010) Eric J. Huang et al.   BRAIN PATHOLOGY

Metabolic Networks of Longevity

(2010) Riekelt H. Houtkooper et al.   CELL

The mitochondrial UPR - protecting organelle protein homeostasis

(2010) C. M. Haynes et al.   JOURNAL OF CELL SCIENCE

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

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Dimer ribbons of ATP synthase shape the inner mitochondrial membrane

(2008) Mike Strauss et al.   EMBO JOURNAL