TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets
Published 2015 View Full Article
- Home
- Publications
- Publication Search
- Publication Details
Title
TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets
Authors
Keywords
ALS, FTD, TDP, TARDBP, Proteinopathy, C9ORF72
Journal
Neurotherapeutics
Volume 12, Issue 2, Pages 352-363
Publisher
Springer Nature
Online
2015-02-04
DOI
10.1007/s13311-015-0338-x
References
Ask authors/readers for more resources
Related references
Note: Only part of the references are listed.- C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration
- (2014) Stephanie May et al. ACTA NEUROPATHOLOGICA
- Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions
- (2014) Johnathan Cooper-Knock et al. BRAIN
- Potentiated Hsp104 Variants Antagonize Diverse Proteotoxic Misfolding Events
- (2014) Meredith E. Jackrel et al. CELL
- Parkin-mediated reduction of nuclear and soluble TDP-43 reverses behavioral decline in symptomatic mice
- (2014) Chen Wenqiang et al. HUMAN MOLECULAR GENETICS
- Misfolded Polyglutamine, Polyalanine, and Superoxide Dismutase 1 Aggregate via Distinct Pathways in the Cell
- (2014) Saskia Polling et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- UBE2E Ubiquitin-conjugating Enzymes and Ubiquitin Isopeptidase Y Regulate TDP-43 Protein Ubiquitination
- (2014) Friederike Hans et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Metabolism and mis-metabolism of the neuropathological signature protein TDP-43
- (2014) C.-C. Huang et al. JOURNAL OF CELL SCIENCE
- Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species
- (2014) E. L. Scotter et al. JOURNAL OF CELL SCIENCE
- Protein Kinase CK-1 Inhibitors As New Potential Drugs for Amyotrophic Lateral Sclerosis
- (2014) Irene G. Salado et al. JOURNAL OF MEDICINAL CHEMISTRY
- A role for human brain pericytes in neuroinflammation
- (2014) Deidre Jansson et al. Journal of Neuroinflammation
- ALS-Linked Mutations Enlarge TDP-43-Enriched Neuronal RNA Granules in the Dendritic Arbor
- (2014) L. Liu-Yesucevitz et al. JOURNAL OF NEUROSCIENCE
- Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models
- (2014) Sami J Barmada et al. Nature Chemical Biology
- Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis
- (2014) Janel O Johnson et al. NATURE NEUROSCIENCE
- Discovery of a Biomarker and Lead Small Molecules to Target r(GGGGCC)-Associated Defects in c9FTD/ALS
- (2014) Zhaoming Su et al. NEURON
- Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations
- (2014) Nael H. Alami et al. NEURON
- Exome-wide Rare Variant Analysis Identifies TUBA4A Mutations Associated with Familial ALS
- (2014) Bradley N. Smith et al. NEURON
- Allele-Specific Knockdown of ALS-Associated Mutant TDP-43 in Neural Stem Cells Derived from Induced Pluripotent Stem Cells
- (2014) Agnes L. Nishimura et al. PLoS One
- Casein Kinase II Induced Polymerization of Soluble TDP-43 into Filaments Is Inhibited by Heat Shock Proteins
- (2014) Yari Carlomagno et al. PLoS One
- Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis
- (2014) C. Yang et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins
- (2014) S. Mizielinska et al. SCIENCE
- Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells
- (2014) I. Kwon et al. SCIENCE
- Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72
- (2014) Yvonne S Davidson et al. Acta Neuropathologica Communications
- Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations
- (2013) Ian R. Mackenzie et al. ACTA NEUROPATHOLOGICA
- C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci
- (2013) Sarah Mizielinska et al. ACTA NEUROPATHOLOGICA
- Stages of pTDP-43 pathology in amyotrophic lateral sclerosis
- (2013) Johannes Brettschneider et al. ANNALS OF NEUROLOGY
- RNA dysfunction and aggrephagy at the centre of an amyotrophic lateral sclerosis/frontotemporal dementia disease continuum
- (2013) Matthew Thomas et al. BRAIN
- Loss of TDP-43 causes age-dependent progressive motor neuron degeneration
- (2013) Yohei Iguchi et al. BRAIN
- ALS/FTLD-linked TDP-43 regulates neurite morphology and cell survival in differentiated neurons
- (2013) Jeong-Ho Han et al. EXPERIMENTAL CELL RESEARCH
- Loss and gain of Drosophila TDP-43 impair synaptic efficacy and motor control leading to age-related neurodegeneration by loss-of-function phenotypes
- (2013) Danielle C. Diaper et al. HUMAN MOLECULAR GENETICS
- TDP-43 Phosphorylation by casein kinase Iε promotes oligomerization and enhances toxicity in vivo
- (2013) Darshana K. Choksi et al. HUMAN MOLECULAR GENETICS
- Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones
- (2013) Maria Udan-Johns et al. HUMAN MOLECULAR GENETICS
- Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLD
- (2013) Danielle C. Diaper et al. HUMAN MOLECULAR GENETICS
- TMPyP4 Porphyrin Distorts RNA G-quadruplex Structures of the Disease-associated r(GGGGCC)nRepeat of theC9orf72Gene and Blocks Interaction of RNA-binding Proteins
- (2013) Bita Zamiri et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study
- (2013) Timothy M Miller et al. LANCET NEUROLOGY
- Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS
- (2013) Hong Joo Kim et al. NATURE
- Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models
- (2013) Hyung-Jun Kim et al. NATURE GENETICS
- State of play in amyotrophic lateral sclerosis genetics
- (2013) Alan E Renton et al. NATURE NEUROSCIENCE
- Targeted high-throughput sequencing identifies a TARDBP mutation as a cause of early-onset FTD without motor neuron disease
- (2013) Matthis Synofzik et al. NEUROBIOLOGY OF AGING
- Unconventional Translation of C9ORF72 GGGGCC Expansion Generates Insoluble Polypeptides Specific to c9FTD/ALS
- (2013) Peter E.A. Ash et al. NEURON
- RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention
- (2013) Christopher J. Donnelly et al. NEURON
- Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis
- (2013) Shuo-Chien Ling et al. NEURON
- Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy
- (2013) A. Serio et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration
- (2013) C. Lagier-Tourenne et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43
- (2013) E. S. Arnold et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS
- (2013) K. Mori et al. SCIENCE
- Hexanucleotide Repeats in ALS/FTD Form Length-Dependent RNA Foci, Sequester RNA Binding Proteins, and Are Neurotoxic
- (2013) Youn-Bok Lee et al. Cell Reports
- The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons
- (2013) Daiyu Honda et al. FEBS Open Bio
- Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72
- (2013) David MA Mann et al. Acta Neuropathologica Communications
- Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion
- (2012) Johannes Brettschneider et al. ACTA NEUROPATHOLOGICA
- The molecular basis of the frontotemporal lobar degeneration–amyotrophic lateral sclerosis spectrum
- (2012) Tim van Langenhove et al. ANNALS OF MEDICINE
- C9ORF72 Repeat Expansion in Amyotrophic Lateral Sclerosis in the Kii Peninsula of Japan
- (2012) Hiroyuki Ishiura et al. ARCHIVES OF NEUROLOGY
- Role of selected mutations in the Q/N rich region of TDP-43 in EGFP-12xQ/N-induced aggregate formation
- (2012) Mauricio Budini et al. BRAIN RESEARCH
- The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease
- (2012) Oliver D. King et al. BRAIN RESEARCH
- Spliceosome integrity is defective in the motor neuron diseases ALS and SMA
- (2012) Hitomi Tsuiji et al. EMBO Molecular Medicine
- ALSoD: A user-friendly online bioinformatics tool for amyotrophic lateral sclerosis genetics
- (2012) Olubunmi Abel et al. HUMAN MUTATION
- Accelerated Disease Onset with Stabilized Familial Amyotrophic Lateral Sclerosis (ALS)-linked Mutant TDP-43 Proteins
- (2012) Shoji Watanabe et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Parkin Ubiquitinates Tar-DNA Binding Protein-43 (TDP-43) and Promotes Its Cytosolic Accumulation via Interaction with Histone Deacetylase 6 (HDAC6)
- (2012) Michaeline L. Hebron et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- 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
- Targeted Depletion of TDP-43 Expression in the Spinal Cord Motor Neurons Leads to the Development of Amyotrophic Lateral Sclerosis-like Phenotypes in Mice
- (2012) Lien-Szu Wu et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study
- (2012) Elisa Majounie et al. LANCET NEUROLOGY
- Does a loss of TDP-43 function cause neurodegeneration?
- (2012) Zuo-Shang Xu Molecular Neurodegeneration
- Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs
- (2012) Clotilde Lagier-Tourenne et al. NATURE NEUROSCIENCE
- Screening of the TARDBP gene in familial and sporadic amyotrophic lateral sclerosis patients of Chinese origin
- (2012) Zhang-Yu Zou et al. NEUROBIOLOGY OF AGING
- The Aggregation and Neurotoxicity of TDP-43 and Its ALS-Associated 25 kDa Fragment Are Differentially Affected by Molecular Chaperones in Drosophila
- (2012) Jenna M. Gregory et al. PLoS One
- Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability
- (2012) B. Bilican et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43
- (2012) I.-F. Wang et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS
- (2011) Safa Al-Sarraj et al. ACTA NEUROPATHOLOGICA
- SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
- (2011) Faisal Fecto ARCHIVES OF NEUROLOGY
- Aggregation of the 35-kDa fragment of TDP-43 causes formation of cytoplasmic inclusions and alteration of RNA processing
- (2011) Mei-Xia Che et al. FASEB JOURNAL
- TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1
- (2011) Karli K. McDonald et al. HUMAN MOLECULAR GENETICS
- Regulation of Autophagy by Neuropathological Protein TDP-43
- (2011) Jayarama Krishnan Bose et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- A “Two-hit” Hypothesis for Inclusion Formation by Carboxyl-terminal Fragments of TDP-43 Protein Linked to RNA Depletion and Impaired Microtubule-dependent Transport
- (2011) G. Scott Pesiridis et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- TDP-43-induced Death Is Associated with Altered Regulation of BIM and Bcl-xL and Attenuated by Caspase-mediated TDP-43 Cleavage
- (2011) Hiroaki Suzuki et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice
- (2011) Lionel M. Igaz et al. JOURNAL OF CLINICAL INVESTIGATION
- C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress
- (2011) Jodi Meyerowitz et al. Molecular Neurodegeneration
- Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia
- (2011) Han-Xiang Deng et al. NATURE
- Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43
- (2011) Magdalini Polymenidou et al. NATURE NEUROSCIENCE
- Characterizing the RNA targets and position-dependent splicing regulation by TDP-43
- (2011) James R Tollervey et al. NATURE NEUROSCIENCE
- Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies
- (2011) Yohei Iguchi et al. NEUROBIOLOGY OF DISEASE
- Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
- (2011) Mariely DeJesus-Hernandez et al. NEURON
- A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
- (2011) Alan E. Renton et al. NEURON
- An MND/ALS phenotype associated withC9orf72repeat expansion: Abundant p62-positive, TDP-43-negative inclusions in cerebral cortex, hippocampus and cerebellum but without associated cognitive decline
- (2011) Claire Troakes et al. NEUROPATHOLOGY
- The C-Terminal TDP-43 Fragments Have a High Aggregation Propensity and Harm Neurons by a Dominant-Negative Mechanism
- (2011) Chunxing Yang et al. PLoS One
- Cytoplasmic Accumulation and Aggregation of TDP-43 upon Proteasome Inhibition in Cultured Neurons
- (2011) Janet van Eersel et al. PLoS One
- Hyperphosphorylation as a Defense Mechanism to Reduce TDP-43 Aggregation
- (2011) Huei-Ying Li et al. PLoS One
- Phosphorylation of the Autophagy Receptor Optineurin Restricts Salmonella Growth
- (2011) P. Wild et al. SCIENCE
- TDP-43 and FUS: a nuclear affair
- (2011) Dorothee Dormann et al. TRENDS IN NEUROSCIENCES
- Cytoplasmic accumulation of TDP-43 in circulating lymphomonocytes of ALS patients with and without TARDBP mutations
- (2010) Giovanni De Marco et al. ACTA NEUROPATHOLOGICA
- Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis
- (2010) Brian C. Kraemer et al. ACTA NEUROPATHOLOGICA
- Enlarging clinical spectrum of FALS with TARDBP gene mutations: S393L variant in an Italian family showing phenotypic variability and relevance for genetic counselling
- (2010) Paola Origone et al. Amyotrophic Lateral Sclerosis
- A role of small heat shock protein B8 (HspB8) in the autophagic removal of misfolded proteins responsible for neurodegenerative diseases
- (2010) Valeria Crippa et al. Autophagy
- VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD
- (2010) Emilie Tresse et al. Autophagy
- Association between novel TARDBP mutations and Chinese patients with amyotrophic lateral sclerosis
- (2010) Hui-Ling Xiong et al. BMC Medical Genetics
- TDP-43 regulates its mRNA levels through a negative feedback loop
- (2010) Youhna M Ayala et al. EMBO JOURNAL
- ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import
- (2010) Dorothee Dormann et al. EMBO JOURNAL
- Neurotoxic effects of TDP-43 overexpression in C. elegans
- (2010) Peter E.A. Ash et al. HUMAN MOLECULAR GENETICS
- Interaction with Polyglutamine Aggregates Reveals a Q/N-rich Domain in TDP-43
- (2010) Rodrigo A. Fuentealba et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion
- (2010) Naoki Suzuki et al. JOURNAL OF HUMAN GENETICS
- Regulation of TDP-43 aggregation by phosphorylation andp62/SQSTM1
- (2010) Owen A. Brady et al. JOURNAL OF NEUROCHEMISTRY
- Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis
- (2010) S. J. Barmada et al. JOURNAL OF NEUROSCIENCE
- Multiplex SILAC Analysis of a Cellular TDP-43 Proteinopathy Model Reveals Protein Inclusions Associated with SUMOylation and Diverse Polyubiquitin Chains
- (2010) Nicholas T. Seyfried et al. MOLECULAR & CELLULAR PROTEOMICS
- Enhancement of proteasome activity by a small-molecule inhibitor of USP14
- (2010) Byung-Hoon Lee et al. NATURE
- Mutations of optineurin in amyotrophic lateral sclerosis
- (2010) Hirofumi Maruyama et al. NATURE
- Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS
- (2010) Janel O. Johnson et al. NEURON
- TDP-43-Mediated Neuron Loss In Vivo Requires RNA-Binding Activity
- (2010) Aaron Voigt et al. PLoS One
- 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
- ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS
- (2010) S.-C. Ling et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- 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
- TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration
- (2010) Hans Wils et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Transgenic Rat Model of Neurodegeneration Caused by Mutation in the TDP Gene
- (2010) Hongxia Zhou et al. PLoS Genetics
- Multiple system degeneration with basophilic inclusions in Japanese ALS patients with FUS mutation
- (2009) Takahisa Tateishi et al. ACTA NEUROPATHOLOGICA
- Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies
- (2009) Manuela Neumann et al. ACTA NEUROPATHOLOGICA
- ALS in Italian professional soccer players: The risk is still present and could be soccer-specific
- (2009) Adriano Chiò et al. Amyotrophic Lateral Sclerosis
- Identification of casein kinase-1 phosphorylation sites on TDP-43
- (2009) Fuyuki Kametani et al. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
- Autophagy: links with the proteasome
- (2009) Trond Lamark et al. CURRENT OPINION IN CELL BIOLOGY
- PLIC proteins or ubiquilins regulate autophagy-dependent cell survival during nutrient starvation
- (2009) Elsa-Noah N'Diaye et al. EMBO REPORTS
- TARDBP(TDP-43) sequence analysis in patients with familial and sporadic ALS: identification of two novel mutations
- (2009) R. Del Bo et al. EUROPEAN JOURNAL OF NEUROLOGY
- Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43
- (2009) Takashi Nonaka et al. HUMAN MOLECULAR GENETICS
- Identification of novel susceptibility loci for Guam neurodegenerative disease: challenges of genome scans in genetic isolates
- (2009) Weiva Sieh et al. HUMAN MOLECULAR GENETICS
- Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo
- (2009) Edor Kabashi et al. HUMAN MOLECULAR GENETICS
- High frequency ofTARDBPgene mutations in Italian patients with amyotrophic lateral sclerosis
- (2009) Lucia Corrado et al. HUMAN MUTATION
- TDP-43 Is a Developmentally Regulated Protein Essential for Early Embryonic Development
- (2009) Chantelle F. Sephton et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity
- (2009) Brian S. Johnson et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Rapamycin Rescues TDP-43 Mislocalization and the Associated Low Molecular Mass Neurofilament Instability
- (2009) Antonella Caccamo et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease
- (2009) Jeong-Sun Ju et al. JOURNAL OF CELL BIOLOGY
- Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin
- (2009) Dorothee Dormann et al. JOURNAL OF NEUROCHEMISTRY
- TDP-43 is recruited to stress granules in conditions of oxidative insult
- (2009) Claudia Colombrita et al. JOURNAL OF NEUROCHEMISTRY
- TARDBP in amyotrophic lateral sclerosis: identification of a novel variant but absence of copy number variation
- (2009) D Baumer et al. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
- TDP-43 M311V mutation in familial amyotrophic lateral sclerosis
- (2009) R Lemmens et al. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
- Broad clinical phenotypes associated with TAR-DNA binding protein (TARDBP) mutations in amyotrophic lateral sclerosis
- (2009) Janine Kirby et al. NEUROGENETICS
- TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations
- (2009) Satomi Maekawa et al. NEUROPATHOLOGY
- Degradation of TDP-43 and its pathogenic form by autophagy and the ubiquitin-proteasome system
- (2009) Xiaoju Wang et al. NEUROSCIENCE LETTERS
- Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity
- (2009) Y.-J. Zhang et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
- (2009) C. Vance et al. SCIENCE
- Enrichment of C-Terminal Fragments in TAR DNA-Binding Protein-43 Cytoplasmic Inclusions in Brain but not in Spinal Cord of Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
- (2008) Lionel M. Igaz et al. AMERICAN JOURNAL OF PATHOLOGY
- A systematic review of antioxidant treatment for amyotrophic lateral sclerosis/motor neuron disease
- (2008) Richard W. Orrell et al. Amyotrophic Lateral Sclerosis
- TDP-43mutation in familial amyotrophic lateral sclerosis
- (2008) Akio Yokoseki et al. ANNALS OF NEUROLOGY
- Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
- (2008) Masato Hasegawa et al. ANNALS OF NEUROLOGY
- Two German Kindreds With Familial Amyotrophic Lateral Sclerosis Due to TARDBP Mutations
- (2008) Peter Kühnlein et al. ARCHIVES OF NEUROLOGY
- Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS
- (2008) Yuki Inukai et al. FEBS LETTERS
- Disturbance of Nuclear and Cytoplasmic TAR DNA-binding Protein (TDP-43) Induces Disease-like Redistribution, Sequestration, and Aggregate Formation
- (2008) Matthew J. Winton et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis
- (2008) Vivianna M Van Deerlin et al. LANCET NEUROLOGY
- TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
- (2008) Edor Kabashi et al. NATURE GENETICS
- TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
- (2008) J. Sreedharan et al. SCIENCE
- Novel Mutations in TARDBP (TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis
- (2008) Nicola J. Rutherford et al. PLoS Genetics
Become a Peeref-certified reviewer
The Peeref Institute provides free reviewer training that teaches the core competencies of the academic peer review process.
Get StartedAsk a Question. Answer a Question.
Quickly pose questions to the entire community. Debate answers and get clarity on the most important issues facing researchers.
Get Started