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Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs
Clotilde Lagier-Tourenne et al.
NATURE NEUROSCIENCE (2012)
Mutant TDP-43 and FUS Cause Age-Dependent Paralysis and Neurodegeneration in C. elegans
Alexandra Vaccaro et al.
PLOS ONE (2012)
Knockdown of the Drosophila Fused in Sarcoma (FUS) Homologue Causes Deficient Locomotive Behavior and Shortening of Motoneuron Terminal Branches
Hiroshi Sasayama et al.
PLOS ONE (2012)
Modeling ALS and FTLD proteinopathies in yeast: An efficient approach for studying protein aggregation and toxicity
Dmitry Kryndushkin et al.
Prion (2012)
TDP-1/TDP-43 Regulates Stress Signaling and Age-Dependent Proteotoxicity in Caenorhabditis elegans
Alexandra Vaccaro et al.
PLOS GENETICS (2012)
A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43
Nicholas A. Lanson et al.
HUMAN MOLECULAR GENETICS (2011)
Wild-type and A315T mutant TDP-43 exert differential neurotoxicity in a Drosophila model of ALS
Patricia S. Estes et al.
HUMAN MOLECULAR GENETICS (2011)
The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span
Ji-Wu Wang et al.
JOURNAL OF CLINICAL INVESTIGATION (2011)
Degenerative Myelopathy in a Bernese Mountain Dog with a Novel SOD1 Missense Mutation
F.A. Wininger et al.
JOURNAL OF VETERINARY INTERNAL MEDICINE (2011)
Nuclear localization sequence of FUS and induction of stress granules by ALS mutants
Jozsef Gal et al.
NEUROBIOLOGY OF AGING (2011)
Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
Mariely DeJesus-Hernandez et al.
NEURON (2011)
A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
Alan E. Renton et al.
NEURON (2011)
Neuronal Function and Dysfunction of Drosophila dTDP
Meng-Jau Lin et al.
PLOS ONE (2011)
Non-ATG-initiated translation directed by microsatellite expansions
Tao Zu et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
A Yeast Model of FUS/TLS-Dependent Cytotoxicity
Shulin Ju et al.
PLOS BIOLOGY (2011)
Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS
Zhihui Sun et al.
PLOS BIOLOGY (2011)
FUS and TARDBP but Not SOD1 Interact in Genetic Models of Amyotrophic Lateral Sclerosis
Edor Kabashi et al.
PLOS GENETICS (2011)
Expression of human FUS protein in Drosophila leads to progressive neurodegeneration
Yanbo Chen et al.
PROTEIN & CELL (2011)
The Q System: A Repressible Binary System for Transgene Expression, Lineage Tracing, and Mosaic Analysis
Christopher J. Potter et al.
CELL (2010)
A genetic model of amyotrophic lateral sclerosis in zebrafish displays phenotypic hallmarks of motoneuron disease
Tennore Ramesh et al.
DISEASE MODELS & MECHANISMS (2010)
Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo
Edor Kabashi et al.
HUMAN MOLECULAR GENETICS (2010)
Neurotoxic effects of TDP-43 overexpression in C-elegans
Peter E. A. Ash et al.
HUMAN MOLECULAR GENETICS (2010)
Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules
Daryl A. Bosco et al.
HUMAN MOLECULAR GENETICS (2010)
Ubiquilin Modifies TDP-43 Toxicity in a Drosophila Model of Amyotrophic Lateral Sclerosis (ALS)
Keith A. Hanson et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2010)
FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis
Ian P. Blair et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2010)
Phosphorylation Promotes Neurotoxicity in a Caenorhabditis elegans Model of TDP-43 Proteinopathy
Nicole F. Liachko et al.
JOURNAL OF NEUROSCIENCE (2010)
TLS Inhibits RNA Polymerase III Transcription
Adelene Y. Tan et al.
MOLECULAR AND CELLULAR BIOLOGY (2010)
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
Andrew C. Elden et al.
NATURE (2010)
Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS
Jane O. Johnson et al.
NEURON (2010)
Spt4/5 stimulates transcription elongation through the RNA polymerase clamp coiled-coil motif
Angela Hirtreiter et al.
NUCLEIC ACIDS RESEARCH (2010)
Connectivity-driven white matter scaling and folding in primate cerebral cortex
Suzana Herculano-Houzel et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
A Drosophila model for TDP-43 proteinopathy
Yan Li et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
Aggregation of Copper-Zinc Superoxide Dismutase in Familial and Sporadic ALS
Madhuri Chattopadhyay et al.
ANTIOXIDANTS & REDOX SIGNALING (2009)
Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS)
Kathryn Volkening et al.
BRAIN RESEARCH (2009)
Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior
Fabian Feiguin et al.
FEBS LETTERS (2009)
TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity
Brian S. Johnson et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
Hristelina Ilieva et al.
JOURNAL OF CELL BIOLOGY (2009)
Generation of transgenic non-human primates with germline transmission
Erika Sasaki et al.
NATURE (2009)
Genome-wide association analysis reveals a SOD1 mutation in canine degenerative myelopathy that resembles amyotrophic lateral sclerosis
Tomoyuki Awano et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
Caroline Vance et al.
SCIENCE (2009)
Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis
T. J. Kwiatkowski et al.
SCIENCE (2009)
An ALS-Linked Mutant SOD1 Produces a Locomotor Defect Associated with Aggregation and Synaptic Dysfunction When Expressed in Neurons of Caenorhabditis elegans
Jiou Wang et al.
PLOS GENETICS (2009)
A Drosophila model for amyotrophic lateral sclerosis reveals motor neuron damage by human SOD1
Melanie R. Watson et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2008)
TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology:: a genetic and histopathological analysis
Vivianna M. Van Deerlin et al.
LANCET NEUROLOGY (2008)
Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription
Xiangting Wang et al.
NATURE (2008)
Mutant SOD1 in cell types other than motor neurons and oligodendrocytes accelerates onset of disease in ALS mice
Koji Yamanaka et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDR-43 aggregation and cellular toxicity
Brian S. Johnson et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
Jemeen Sreedharan et al.
SCIENCE (2008)
Establishment of a cell model of ALS disease: Golgi apparatus disruption occurs independently from apoptosis
Catarina Gomes et al.
BIOTECHNOLOGY LETTERS (2008)
Induction of pluripotent stem cells from adult human fibroblasts by defined factors
Kazutoshi Takahashi et al.
CELL (2007)
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Tetsuaki Arai et al.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2006)
Motor neuron disease in mice expressing the wild type-like D90A mutant superoxide dismutase-1
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JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2006)
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Manuela Neumann et al.
SCIENCE (2006)
ALS:: A disease of motor neurons and their nonneuronal neighbors
Sverine Boillee et al.
NEURON (2006)
Familial ALS-superoxide dismutases associate with mitochondria and shift their redox potentials
Alberto Ferri et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2006)
Genetic mosaic with dual binary transcriptional systems in Drosophila
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NATURE NEUROSCIENCE (2006)
Low levels of ALS-linked Cu/Zn superoxide dismutase increase the production of reactive oxygen species and cause mitochondrial damage and death in motor neuron-like cells
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JOURNAL OF THE NEUROLOGICAL SCIENCES (2005)
Impairment of glutamate transport and increased vulnerability to oxidative stress in neuroblastoma SH-SY5Y cells expressing a Cu,Zn superoxide dismutase typical of familial amyotrophic lateral sclerosis
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NEUROCHEMISTRY INTERNATIONAL (2005)
The Microprocessor complex mediates the genesis of microRNAs
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NATURE (2004)
Analysis of the cytosolic proteome in a cell culture model of familial amyotrophic lateral sclerosis reveals alterations to the proteasome, antioxidant defenses, and nitric oxide synthetic pathways
S Allen et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2003)
Cu/Zn superoxide dismutase (SOD1) mutations associated with familial amyotrophic lateral sclerosis (ALS) affect cellular free radical release in the presence of oxidative stress
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AMYOTROPHIC LATERAL SCLEROSIS AND OTHER MOTOR NEURON DISORDERS (2002)
Characterization and functional implications of the RNA binding properties of nuclear factor TDP-43, a novel splicing regulator of CFTR exon 9
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JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic Caenorhabditis elegans
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HUMAN MOLECULAR GENETICS (2001)
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