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UBE2E Ubiquitin-conjugating Enzymes and Ubiquitin Isopeptidase Y Regulate TDP-43 Protein Ubiquitination
Friederike Hans et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2014)
Futsch/MAP1B mRNA Is a Translational Target of TDP-43 and Is Neuroprotective in a Drosophila Model of Amyotrophic Lateral Sclerosis
Alyssa N. Coyne et al.
JOURNAL OF NEUROSCIENCE (2014)
Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models
Hyung-Jun Kim et al.
NATURE GENETICS (2014)
Reduced C9orf72 protein levels in frontal cortex of amyotrophic lateral sclerosis and frontotemporal degeneration brain with the C9ORF72 hexanucleotide repeat expansion
Adrian J. Waite et al.
NEUROBIOLOGY OF AGING (2014)
Aggregate formation prevents dTDP-43 neurotoxicity in Drosophila melanogaster eye
Lucia Cragnaz et al.
NEUROBIOLOGY OF DISEASE (2014)
Chronological requirements of TDP-43 function in synaptic organization and locomotive control
Giulia Romano et al.
NEUROBIOLOGY OF DISEASE (2014)
Antisense Proline-Arginine RAN Dipeptides Linked to C9ORF72-ALS/FTD Form Toxic Nuclear Aggregates that Initiate In Vitro and In Vivo Neuronal Death
Xinmei Wen et al.
NEURON (2014)
Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations
Nael H. Alami et al.
NEURON (2014)
Lipoic acid – biological activity and therapeutic potential
Anna Gorąca et al.
Pharmacological Reports (2014)
C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins
Sarah Mizielinska et al.
SCIENCE (2014)
An ALS-associated mutation in the FUS 3′-UTR disrupts a microRNA-FUS regulatory circuitry
Stefano Dini Modigliani et al.
NATURE COMMUNICATIONS (2014)
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Katarzyna Miskiewicz et al.
CELL REPORTS (2014)
piRNA clusters and open chromatin structure
Soichiro Yamanaka et al.
MOBILE DNA (2014)
Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins
Kohji Mori et al.
ACTA NEUROPATHOLOGICA (2013)
Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS
Tania F. Gendron et al.
ACTA NEUROPATHOLOGICA (2013)
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Veronique V. Belzil et al.
ACTA NEUROPATHOLOGICA (2013)
Loss of Function of C9orf72 Causes Motor Deficits in a Zebrafish Model of Amyotrophic Lateral Sclerosis
Sorana Ciura et al.
ANNALS OF NEUROLOGY (2013)
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Sonja Koerner et al.
BMC NEUROLOGY (2013)
Muscleblind, BSF and TBPH are mislocalized in the muscle sarcomere of a Drosophila myotonic dystrophy model
Beatriz Llamusi et al.
DISEASE MODELS & MECHANISMS (2013)
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Patricia S. Estes et al.
DISEASE MODELS & MECHANISMS (2013)
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Ayan Banerjee et al.
FEBS JOURNAL (2013)
Piwi induces piRNA-guided transcriptional silencing and establishment of a repressive chromatin state
Adrien Le Thomas et al.
GENES & DEVELOPMENT (2013)
Protein disulfide isomerase in ALS mouse glia links protein misfolding with NADPH oxidase-catalyzed superoxide production
Merja Jaronen et al.
HUMAN MOLECULAR GENETICS (2013)
The FTD/ALS-associated RNA-binding protein TDP-43 regulates the robustness of neuronal specification through microRNA-9a in Drosophila
Zhaodong Li et al.
HUMAN MOLECULAR GENETICS (2013)
RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations
J. Gavin Daigle et al.
HUMAN MOLECULAR GENETICS (2013)
Loss and gain of Drosophila TDP-43 impair synaptic efficacy and motor control leading to age-related neurodegeneration by loss-of-function phenotypes
Danielle C. Diaper et al.
HUMAN MOLECULAR GENETICS (2013)
RNA binding mediates neurotoxicity in the transgenic Drosophila model of TDP-43 proteinopathy
Ryoko Ihara et al.
HUMAN MOLECULAR GENETICS (2013)
Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLD
Danielle C. Diaper et al.
HUMAN MOLECULAR GENETICS (2013)
Mutations in the 3′ untranslated region of FUS causing FUS overexpression are associated with amyotrophic lateral sclerosis
Mario Sabatelli et al.
HUMAN MOLECULAR GENETICS (2013)
Stress granules as crucibles of ALS pathogenesis
Yun R. Li et al.
JOURNAL OF CELL BIOLOGY (2013)
Immunodetection of Disease-Associated Conformers of Mutant Cu/Zn Superoxide Dismutase 1 Selectively Expressed in Degenerating Neurons in Amyotrophic Lateral Sclerosis
Javier Sabado et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2013)
Defects in Synapse Structure and Function Precede Motor Neuron Degeneration in Drosophila Models of FUS-Related ALS
Mohammad Shahidullah et al.
JOURNAL OF NEUROSCIENCE (2013)
Oligodendrocyte-Specific Activation of PERK Signaling Protects Mice against Experimental Autoimmune Encephalomyelitis
Wensheng Lin et al.
JOURNAL OF NEUROSCIENCE (2013)
Cognitive changes predict functional decline in ALS A population-based longitudinal study
Marwa Elamin et al.
NEUROLOGY (2013)
Identification of Genetic Modifiers of TDP-43 Neurotoxicity in Drosophila
Lihong Zhan et al.
PLOS ONE (2013)
Deletion of C9ORF72 Results in Motor Neuron Degeneration and Stress Sensitivity in C-elegans
Martine Therrien et al.
PLOS ONE (2013)
Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration
Zihui Xu et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2013)
Transposition-Driven Genomic Heterogeneity in the Drosophila Brain
Paola N. Perrat et al.
SCIENCE (2013)
The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS
Kohji Mori et al.
SCIENCE (2013)
TDP-43 Loss-of-Function Causes Neuronal Loss Due to Defective Steroid Receptor-Mediated Gene Program Switching in Drosophila
Lies Vanden Broeck et al.
CELL REPORTS (2013)
RNA Seeds Higher-Order Assembly of FUS Protein
Jacob C. Schwartz et al.
CELL REPORTS (2013)
ALS-Associated FUS Mutations Result in Compromised FUS Alternative Splicing and Autoregulation
Yueqin Zhou et al.
PLOS GENETICS (2013)
Misfolded SOD1 and ALS: Zeroing in on mitochondria
Sarah Pickles et al.
AMYOTROPHIC LATERAL SCLEROSIS (2012)
Cell-free Formation of RNA Granules: Low Complexity Sequence Domains Form Dynamic Fibers within Hydrogels
Masato Kato et al.
CELL (2012)
Cell-free Formation of RNA Granules: Bound RNAs Identify Features and Components of Cellular Assemblies
Tina W. Han et al.
CELL (2012)
Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS
Dorothee Dormann et al.
EMBO JOURNAL (2012)
Evolutionarily conserved protein arginine methyltransferases in non-mammalian animal systems
Yi-Chun Wang et al.
FEBS JOURNAL (2012)
High-content RNAi screening identifies the Type 1 inositol triphosphate receptor as a modifier of TDP-43 localization and neurotoxicity
Sang Hwa Kim et al.
HUMAN MOLECULAR GENETICS (2012)
Cellular Model of TAR DNA-binding Protein 43 (TDP-43) Aggregation Based on Its C-terminal Gln/Asn-rich Region
Mauricio Budini et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2012)
The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study
Julie Phukan et al.
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY (2012)
Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study
Elisa Majounie et al.
LANCET NEUROLOGY (2012)
Motor neuron apoptosis and neuromuscular junction perturbation are prominent features in a Drosophila model of Fus-mediated ALS
Ruohan Xia et al.
MOLECULAR NEURODEGENERATION (2012)
Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system
Hemmo Meyer et al.
NATURE CELL BIOLOGY (2012)
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)
Accumulation of insoluble forms of FUS protein correlates with toxicity in Drosophila
Laetitia Miguel et al.
NEUROBIOLOGY OF AGING (2012)
The Aggregation and Neurotoxicity of TDP-43 and Its ALS-Associated 25 kDa Fragment Are Differentially Affected by Molecular Chaperones in Drosophila
Jenna M. Gregory 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)
Ataxin-2-Like Is a Regulator of Stress Granules and Processing Bodies
Christian Kaehler et al.
PLOS ONE (2012)
Activation of the unfolded protein response enhances motor recovery after spinal cord injury
V. Valenzuela et al.
CELL DEATH & DISEASE (2012)
Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain
Boris Rogelj et al.
SCIENTIFIC REPORTS (2012)
Comparison of Parallel High-Throughput RNA Sequencing Between Knockout of TDP-43 and Its Overexpression Reveals Primarily Nonreciprocal and Nonoverlapping Gene Expression Changes in the Central Nervous System of Drosophila
Dennis J. Hazelett et al.
G3-GENES GENOMES GENETICS (2012)
Nuclear Transport Impairment of Amyotrophic Lateral Sclerosis-Linked Mutations in FUS/TLS
Daisuke Ito et al.
ANNALS OF NEUROLOGY (2011)
Understanding the role of TDP-43 and FUS/TLS in ALS and beyond
Sandrine Da Cruz et al.
CURRENT OPINION IN NEUROBIOLOGY (2011)
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)
Mechanisms of mitophagy
Richard J. Youle et al.
NATURE REVIEWS MOLECULAR CELL BIOLOGY (2011)
Both cytoplasmic and nuclear accumulations of the protein are neurotoxic in Drosophila models of TDP-43 proteinopathies
Laetitia Miguel et al.
NEUROBIOLOGY OF DISEASE (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)
The chromodomains of CHD1 are critical for enzymatic activity but less important for chromatin localization
Stefano Morettini et al.
NUCLEIC ACIDS RESEARCH (2011)
Intracellular localization and splicing regulation of FUS/TLS are variably affected by amyotrophic lateral sclerosis-linked mutations
Yoshihiro Kino et al.
NUCLEIC ACIDS RESEARCH (2011)
THE UNFOLDED PROTEIN RESPONSE: INTEGRATING STRESS SIGNALS THROUGH THE STRESS SENSOR IRE1α
Claudio Hetz et al.
PHYSIOLOGICAL REVIEWS (2011)
Neuronal Function and Dysfunction of Drosophila dTDP
Meng-Jau Lin et al.
PLOS ONE (2011)
A yeast functional screen predicts new candidate ALS disease genes
Julien Couthouis et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation
Peter Walter et al.
SCIENCE (2011)
Biogenesis and function of nuclear bodies
Yuntao S. Mao et al.
TRENDS IN GENETICS (2011)
Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS
Zhihui Sun et al.
PLOS BIOLOGY (2011)
The ISWI Chromatin Remodeler Organizes the hsrω ncRNA-Containing Omega Speckle Nuclear Compartments
Maria C. Onorati et al.
PLOS GENETICS (2011)
FUS and TARDBP but Not SOD1 Interact in Genetic Models of Amyotrophic Lateral Sclerosis
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PLOS GENETICS (2011)
FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration
Cao Huang et al.
PLOS GENETICS (2011)
Expression of human FUS protein in Drosophila leads to progressive neurodegeneration
Yanbo Chen et al.
PROTEIN & CELL (2011)
Impaired glucose tolerance in patients with amyotrophic lateral sclerosis
Pierre-Francois Pradat et al.
AMYOTROPHIC LATERAL SCLEROSIS (2010)
Nuclear functions of the HMG proteins
Raymond Reeves
BIOCHIMICA ET BIOPHYSICA ACTA-GENE REGULATORY MECHANISMS (2010)
Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6
Fabienne C. Fiesel et al.
EMBO JOURNAL (2010)
ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import
Dorothee Dormann et al.
EMBO JOURNAL (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)
Amyotrophic Lateral Sclerosis-associated Proteins TDP-43 and FUS/TLS Function in a Common Biochemical Complex to Co-regulate HDAC6 mRNA
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JOURNAL OF BIOLOGICAL CHEMISTRY (2010)
Mutations of FUS gene in sporadic amyotrophic lateral sclerosis
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JOURNAL OF MEDICAL GENETICS (2010)
TDP-43 Mediates Degeneration in a Novel Drosophila Model of Disease Caused by Mutations in VCP/p97
Gillian P. Ritson et al.
JOURNAL OF NEUROSCIENCE (2010)
TLS Inhibits RNA Polymerase III Transcription
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MOLECULAR AND CELLULAR BIOLOGY (2010)
Gymnastics of Molecular Chaperones
Matthias P. Mayer
MOLECULAR CELL (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)
TDP-43-Mediated Neuron Loss In Vivo Requires RNA-Binding Activity
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PLOS ONE (2010)
A Drosophila model for TDP-43 proteinopathy
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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
The multiple roles of TDP-43 in pre-mRNA processing and gene expression regulation
Emanuele Buratti et al.
RNA BIOLOGY (2010)
Rethinking ALS: The FUS about TDP-43
Clotilde Lagier-Tourenne et al.
CELL (2009)
Regulation of Organ Size: Insights From the Drosophila Hippo Signaling Pathway
Madhuri Kango-Singh et al.
DEVELOPMENTAL DYNAMICS (2009)
Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior
Fabian Feiguin et al.
FEBS LETTERS (2009)
Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
Hristelina Ilieva et al.
JOURNAL OF CELL BIOLOGY (2009)
Hypermetabolism in ALS patients: an early and persistent phenomenon
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JOURNAL OF NEUROLOGY (2009)
Functional mapping of the interaction between TDP-43 and hnRNP A2 in vivo
Andrea D'Ambrogio et al.
NUCLEIC ACIDS RESEARCH (2009)
Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
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SCIENCE (2009)
Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis
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SCIENCE (2009)
Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching
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MOLECULAR BRAIN (2009)
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ACTA NEUROPATHOLOGICA (2008)
Enhanced Integrated Stress Response Promotes Myelinating Oligodendrocyte Survival in Response to Interferon-γ
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AMERICAN JOURNAL OF PATHOLOGY (2008)
TDP-43 mutation in familial amyotrophic lateral sclerosis
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ANNALS OF NEUROLOGY (2008)
TDP-43 A315T mutation in familial motor neuron disease
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ANNALS OF NEUROLOGY (2008)
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BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2008)
The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress response
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BMC CELL BIOLOGY (2008)
Abnormal phosphorylation of ser409/410 of TDP-43 in FTLD-U and ALS
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FEBS LETTERS (2008)
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
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LANCET NEUROLOGY (2008)
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
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NATURE GENETICS (2008)
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NEUROLOGY (2008)
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SCIENCE (2008)
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis
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SCIENCE (2008)
Novel Mutations in TARDBP(TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis
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PLOS GENETICS (2008)
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ACTA NEUROPATHOLOGICA (2008)
Mitochondrial alterations in dorsal root ganglion cells in sporadic amyotrophic lateral sclerosis
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ACTA NEUROPATHOLOGICA (2007)
Exogenous delivery of heat shock protein 70 increases lifespan in a mouse model of amyotrophic lateral sclerosis
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JOURNAL OF NEUROSCIENCE (2007)
Wild-type superoxide dismutase acquires binding and toxic properties of ALS-linked mutant forms through oxidation
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JOURNAL OF NEUROCHEMISTRY (2007)
Stress-induced retrotranslocation of clusterin/ApoJ into the cytosol
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TRAFFIC (2007)
TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions
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JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2007)
HDAC6 controls major cell response pathways to cytotoxic accumulation of protein aggregates
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GENES & DEVELOPMENT (2007)
Ataxin-2 interacts with the DEAD/H-box RNA helicase DDX6 and interferes with P-bodies and stress granules
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MOLECULAR BIOLOGY OF THE CELL (2007)
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
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BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS (2006)
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NUCLEIC ACIDS RESEARCH (2006)
MicroRNA-9a ensures the precise specification of sensory organ precursors in Drosophila
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GENES & DEVELOPMENT (2006)
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SCIENCE (2006)
Drosophila in the study of neurodegenerative disease
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NEURON (2006)
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CELL (2006)
Bruchpilot promotes active zone assembly, Ca2+ channel clustering, and vesicle release
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SCIENCE (2006)
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JOURNAL OF CELL BIOLOGY (2005)
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INTERNATIONAL JOURNAL OF PHARMACEUTICS (2005)
Human, Drosophila, and C-elegans TDP43:: Nucleic acid binding properties and splicing regulatory function
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JOURNAL OF MOLECULAR BIOLOGY (2005)
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NATURE REVIEWS MOLECULAR CELL BIOLOGY (2005)
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BIOCHEMICAL JOURNAL (2004)
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GENOMICS (2004)
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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)
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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)
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NEUROBIOLOGY OF DISEASE (2002)
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JOURNAL OF NEUROSCIENCE (2002)
ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats
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GENES & DEVELOPMENT (2002)
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MOLECULAR BIOLOGY OF THE CELL (2002)
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NEUROBIOLOGY OF DISEASE (2002)
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NATURE REVIEWS NEUROSCIENCE (2001)
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GENOME RESEARCH (2001)
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NEW ENGLAND JOURNAL OF MEDICINE (2001)
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JOURNAL OF NEUROSCIENCE (2001)
Mutant Cu/Zn-superoxide dismutase proteins have altered solubility and interact with heat shock/stress proteins in models of amyotrophic lateral sclerosis
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JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
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JOURNAL OF BIOLOGICAL CHEMISTRY (2001)
p190 RhoGAP is the principal Src substrate in brain and regulates axon outgrowth, guidance and fasciculation
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NATURE CELL BIOLOGY (2001)
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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2000)
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SCIENCE (2000)
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SCIENCE (2000)
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JOURNAL OF STRUCTURAL BIOLOGY (2000)
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