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A Seeding Reaction Recapitulates Intracellular Formation of Sarkosyl-insoluble Transactivation Response Element (TAR) DNA-binding Protein-43 Inclusions
Yoshiaki Furukawa et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2011)
Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice
Lionel M. Igaz et al.
JOURNAL OF CLINICAL INVESTIGATION (2011)
The Dying-Back Phenomenon of Motor Neurons in ALS
Michal Dadon-Nachum et al.
JOURNAL OF MOLECULAR NEUROSCIENCE (2011)
Regulation of TDP-43 aggregation by phosphorylation andp62/SQSTM1
Owen A. Brady et al.
JOURNAL OF NEUROCHEMISTRY (2011)
TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor
Colleen M. Dewey et al.
MOLECULAR AND CELLULAR BIOLOGY (2011)
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice
Ya-Fei Xu et al.
MOLECULAR NEURODEGENERATION (2011)
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43
Magdalini Polymenidou et al.
NATURE NEUROSCIENCE (2011)
Characterizing the RNA targets and position-dependent splicing regulation by TDP-43
James R. Tollervey et al.
NATURE NEUROSCIENCE (2011)
An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity
Weirui Guo et al.
NATURE STRUCTURAL & MOLECULAR BIOLOGY (2011)
Cytoplasmic Accumulation and Aggregation of TDP-43 upon Proteasome Inhibition in Cultured Neurons
Janet van Eersel et al.
PLOS ONE (2011)
The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation
Peter Walter et al.
SCIENCE (2011)
Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target
Sian C. Barber et al.
FREE RADICAL BIOLOGY AND MEDICINE (2010)
Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U
Kuen-Jer Tsai et al.
JOURNAL OF EXPERIMENTAL MEDICINE (2010)
Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis
Sami J. Barmada et al.
JOURNAL OF NEUROSCIENCE (2010)
Wild-Type Human TDP-43 Expression Causes TDP-43 Phosphorylation, Mitochondrial Aggregation, Motor Deficits, and Early Mortality in Transgenic Mice
Ya-Fei Xu et al.
JOURNAL OF NEUROSCIENCE (2010)
Synergistic Effect Between Proteasome and Autophagosome in the Clearance of Polyubiquitinated TDP-43
Makoto Urushitani et al.
JOURNAL OF NEUROSCIENCE RESEARCH (2010)
Global Analysis of TDP-43 Interacting Proteins Reveals Strong Association with RNA Splicing and Translation Machinery
Brian D. Freibaum et al.
JOURNAL OF PROTEOME RESEARCH (2010)
Multiplex SILAC Analysis of a Cellular TDP-43 Proteinopathy Model Reveals Protein Inclusions Associated with SUMOylation and Diverse Polyubiquitin Chains
Nicholas T. Seyfried et al.
MOLECULAR & CELLULAR PROTEOMICS (2010)
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia
Ian R. A. Mackenzie et al.
LANCET NEUROLOGY (2010)
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
Andrew C. Elden et al.
NATURE (2010)
Progressive motor weakness in transgenic mice expressing human TDP-43
Nancy R. Stallings et al.
NEUROBIOLOGY OF DISEASE (2010)
Phosphorylated and cleaved TDP-43 in ALS, FTLD and other neurodegenerative disorders and in cellular models of TDP-43 proteinopathy
Tetsuaki Arai et al.
NEUROPATHOLOGY (2010)
Degradation of TDP-43 and its pathogenic form by autophagy and the ubiquitin-proteasome system
Xiaoju Wang et al.
NEUROSCIENCE LETTERS (2010)
TDP-43-Mediated Neuron Loss In Vivo Requires RNA-Binding Activity
Aaron Voigt et al.
PLOS ONE (2010)
The C-Terminal TDP-43 Fragments Have a High Aggregation Propensity and Harm Neurons by a Dominant-Negative Mechanism
Chunxing Yang et al.
PLOS ONE (2010)
Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice
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PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies
Manuela Neumann et al.
ACTA NEUROPATHOLOGICA (2009)
TARDBP Mutations in Motoneuron Disease with Frontotemporal Lobar Degeneration
Lina Benajiba et al.
ANNALS OF NEUROLOGY (2009)
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43
Takashi Nonaka et al.
HUMAN MOLECULAR GENETICS (2009)
High Frequency of TARDBP Gene Mutations in Italian Patients With Amyotrophic Lateral Sclerosis
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HUMAN MUTATION (2009)
Mutation within TARDBP Leads to Frontotemporal Dementia without Motor Neuron Disease
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HUMAN MUTATION (2009)
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies
Lionel M. Igaz et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2009)
Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin
Dorothee Dormann et al.
JOURNAL OF NEUROCHEMISTRY (2009)
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration
Iga Wegorzewska et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
TDP-43 mutation in familial amyotrophic lateral sclerosis
Akio Yokoseki et al.
ANNALS OF NEUROLOGY (2008)
Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Masato Hasegawa et al.
ANNALS OF NEUROLOGY (2008)
Abnormal phosphorylation of ser409/410 of TDP-43 in FTLD-U and ALS
Yuki Inukai et al.
FEBS LETTERS (2008)
Autophagy induction and autophagosome clearance in neurons: Relationship to autophagic pathology in Alzheimer's disease
Barry Boland et al.
JOURNAL OF NEUROSCIENCE (2008)
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
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NATURE GENETICS (2008)
Novel Mutations in TARDBP(TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis
Nicola J. Rutherford et al.
PLOS GENETICS (2008)
Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43
Yong-Jie Zhang et al.
JOURNAL OF NEUROSCIENCE (2007)
Glial cells more than support cells?
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INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY (2007)
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
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SCIENCE (2006)
hnRNP A1 relocalization to the stress granules reflects a role in the stress response
Sonia Guil et al.
MOLECULAR AND CELLULAR BIOLOGY (2006)
Mitochondrial dysfunction and its role in motor neuron degeneration in ALS
G Manfredi et al.
MITOCHONDRION (2005)
For whom the bell tolls: protein quality control of the endoplasmic reticulum and the ubiquitin-proteasome connection
Z Kostova et al.
EMBO JOURNAL (2003)
Stress granules: sites of mRNA triage that regulate mRNA stability and translatability
N Kedersha et al.
BIOCHEMICAL SOCIETY TRANSACTIONS (2002)
Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosis
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JOURNAL OF NEUROSCIENCE (2001)
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