FUS Interacts with HSP60 to Promote Mitochondrial Damage

Inhibition of mitochondrial protein import by mutant huntingtin

(2014) Hiroko Yano et al.   NATURE NEUROSCIENCE

The Hsp60 folding machinery is crucial for manganese superoxide dismutase folding and function

(2013) R. Magnoni et al.   FREE RADICAL RESEARCH

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

TARDBPandFUSMutations Associated with Amyotrophic Lateral Sclerosis: Summary and Update

(2013) Serena Lattante et al.   HUMAN MUTATION

Transportin 1 accumulates specifically with FET proteins but no other transportin cargos in FTLD-FUS and is absent in FUS inclusions in ALS with FUS mutations

(2012) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

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

Recent Advances in the Genetics of the ALS-FTLD Complex

(2012) Huw R. Morris et al.   Current Neurology and Neuroscience Reports

How do the RNA-binding proteins TDP-43 and FUS relate to amyotrophic lateral sclerosis and frontotemporal degeneration, and to each other?

(2012) Robert H. Baloh   CURRENT OPINION IN NEUROLOGY

HSP60 as a Drug Target

(2012) Hiroyuki Nakamura et al.   CURRENT PHARMACEUTICAL DESIGN

Loss of fused in sarcoma (FUS) promotes pathological Tau splicing

(2012) Denise Orozco et al.   EMBO REPORTS

Swedish Alzheimer Mutation Induces Mitochondrial Dysfunction Mediated by HSP60 Mislocalization of Amyloid Precursor Protein (APP) and Beta-Amyloid

(2012) Ken Carlson Walls et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

DRP1-dependent mitochondrial fission initiates follicle cell differentiation duringDrosophilaoogenesis

(2012) Kasturi Mitra et al.   JOURNAL OF CELL BIOLOGY

Mitochondria and ALS: Implications from novel genes and pathways

(2012) Mauro Cozzolino et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis

(2012) Christophe Verbeeck et al.   Molecular Neurodegeneration

Molecular pathology and genetic advances in amyotrophic lateral sclerosis: an emerging molecular pathway and the significance of glial pathology

(2011) Paul G. Ince et al.   ACTA NEUROPATHOLOGICA

A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies

(2011) T. Lashley et al.   BRAIN

Understanding the role of TDP-43 and FUS/TLS in ALS and beyond

(2011) Sandrine Da Cruz et al.   CURRENT OPINION IN NEUROBIOLOGY

Mitochondrial oxidative stress causes mitochondrial fragmentation via differential modulation of mitochondrial fission-fusion proteins

(2011) Shengnan Wu et al.   FEBS Journal

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

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

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

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

The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span

(2011) Ji-Wu Wang et al.   JOURNAL OF CLINICAL INVESTIGATION

FUS Immunogold Labeling TEM Analysis of the Neuronal Cytoplasmic Inclusions of Neuronal Intermediate Filament Inclusion Disease: A Frontotemporal Lobar Degeneration with FUS Proteinopathy

(2011) Tristan Page et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

(2011) Wenjun Song et al.   NATURE MEDICINE

Clinical genetics of amyotrophic lateral sclerosis: what do we really know?

(2011) Peter M. Andersen et al.   Nature Reviews Neurology

An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity

(2011) Weirui Guo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset

(2011) Christine Vande Velde et al.   PLoS One

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

FUS and TARDBP but Not SOD1 Interact in Genetic Models of Amyotrophic Lateral Sclerosis

(2011) Edor Kabashi et al.   PLoS Genetics

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

(2011) Cao Huang et al.   PLoS Genetics

FUS/TLS forms cytoplasmic aggregates, inhibits cell growth and interacts with TDP-43 in a yeast model of amyotrophic lateral sclerosis

(2011) Dmitry Kryndushkin et al.   Protein & Cell

Expression of human FUS protein in Drosophila leads to progressive neurodegeneration

(2011) Yanbo Chen et al.   Protein & Cell

Expression of human FUS/TLS in yeast leads to protein aggregation and cytotoxicity, recapitulating key features of FUS proteinopathy

(2011) Kazuo Fushimi et al.   Protein & Cell

RNA-binding proteins in neurodegenerative disease: TDP-43 and beyond

(2011) Keith A. Hanson et al.   Wiley Interdisciplinary Reviews-RNA

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

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

(2010) Dorothee Dormann et al.   EMBO JOURNAL

Drosophila FMRP regulates microtubule network formation and axonal transport of mitochondria

(2010) Aiyu Yao et al.   HUMAN MOLECULAR GENETICS

TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration

(2010) C. Lagier-Tourenne et al.   HUMAN MOLECULAR GENETICS

The mitochondrial UPR - protecting organelle protein homeostasis

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

Wild-Type Human TDP-43 Expression Causes TDP-43 Phosphorylation, Mitochondrial Aggregation, Motor Deficits, and Early Mortality in Transgenic Mice

(2010) Y.-F. Xu et al.   JOURNAL OF NEUROSCIENCE

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

(2010) Ian RA Mackenzie et al.   LANCET NEUROLOGY

Gymnastics of Molecular Chaperones

(2010) Matthias P. Mayer   MOLECULAR CELL

Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations

(2010) D. Baumer et al.   NEUROLOGY

Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue

(2010) Liqun Liu-Yesucevitz et al.   PLoS One

A Drosophila model for TDP-43 proteinopathy

(2010) Y. Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice

(2010) X. Shan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease

(2009) Manuela Neumann et al.   ACTA NEUROPATHOLOGICA

FUS pathology in basophilic inclusion body disease

(2009) David G. Munoz et al.   ACTA NEUROPATHOLOGICA

A new subtype of frontotemporal lobar degeneration with FUS pathology

(2009) M. Neumann et al.   BRAIN

What's the FUS!

(2009) Bryan J Traynor et al.   LANCET NEUROLOGY

Amyloid precursor protein, heat-shock proteins, and Bcl-2 form a complex in mitochondria and modulate mitochondria function and apoptosis in N2a cells

(2009) Ting-Ting Yang et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Midline crossing and Slit responsiveness of commissural axons require USP33

(2009) Junichi Yuasa-Kawada et al.   NATURE NEUROSCIENCE

Two Italian kindreds with familial amyotrophic lateral sclerosis due to FUS mutation

(2009) Adriano Chiò et al.   NEUROBIOLOGY OF AGING

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

Mitochondrial Disorders in the Nervous System

(2008) Salvatore DiMauro et al.   Annual Review of Neuroscience

Hsp60D is essential for caspase-mediated induced apoptosis in Drosophila melanogaster

(2008) Richa Arya et al.   CELL STRESS & CHAPERONES

A novel deletion in the GTPase domain of OPA1 causes defects in mitochondrial morphology and distribution, but not in function

(2008) Marco Spinazzi et al.   HUMAN MOLECULAR GENETICS

A Vicious Cycle Involving Release of Heat Shock Protein 60 from Injured Cells and Activation of Toll-Like Receptor 4 Mediates Neurodegeneration in the CNS

(2008) S. Lehnardt et al.   JOURNAL OF NEUROSCIENCE

Mitochondrial Membrane Potential in Axons Increases with Local Nerve Growth Factor or Semaphorin Signaling

(2008) J. Verburg et al.   JOURNAL OF NEUROSCIENCE

The Expression of Heat Shock Protein HSP60A Reveals a Dynamic Mitochondrial Pattern inDrosophila melanogasterEmbryos

(2008) Luis Alberto Baena-López et al.   JOURNAL OF PROTEOME RESEARCH

A rapid Percoll gradient procedure for preparation of synaptosomes

(2008) Peter R Dunkley et al.   Nature Protocols

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria

(2008) C. Vande Velde et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA