Protein Misdirection Inside and Outside Motor Neurons in Amyotrophic Lateral Sclerosis (ALS): A Possible Clue for Therapeutic Strategies
Published 2011 View Full Article
- Home
- Publications
- Publication Search
- Publication Details
Title
Protein Misdirection Inside and Outside Motor Neurons in Amyotrophic Lateral Sclerosis (ALS): A Possible Clue for Therapeutic Strategies
Authors
Keywords
-
Journal
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 12, Issue 10, Pages 6980-7003
Publisher
MDPI AG
Online
2011-10-19
DOI
10.3390/ijms12106980
References
Ask authors/readers for more resources
Related references
Note: Only part of the references are listed.- Protein Aggregation and Defective RNA Metabolism as Mechanisms for Motor Neuron Damage
- (2012) N. Ticozzi et al. CNS & Neurological Disorders-Drug Targets
- Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments
- (2011) Vivek Swarup et al. BRAIN
- 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
- Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis
- (2011) Adam K. Walker et al. IUBMB LIFE
- Increased Axonal Mitochondrial Mobility Does Not Slow Amyotrophic Lateral Sclerosis (ALS)-like Disease in Mutant SOD1 Mice
- (2011) Yi-Bing Zhu et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- A Seeding Reaction Recapitulates Intracellular Formation of Sarkosyl-insoluble Transactivation Response Element (TAR) DNA-binding Protein-43 Inclusions
- (2011) Yoshiaki Furukawa 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
- 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
- Misfolded superoxide dismutase-1 in CSF from amyotrophic lateral sclerosis patients
- (2011) Per Zetterström et al. JOURNAL OF NEUROCHEMISTRY
- RNA targets of TDP-43 identified by UV-CLIP are deregulated in ALS
- (2011) Shangxi Xiao et al. MOLECULAR AND CELLULAR NEUROSCIENCE
- Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia
- (2011) Han-Xiang Deng et al. NATURE
- Astrocytes from familial and sporadic ALS patients are toxic to motor neurons
- (2011) Amanda M Haidet-Phillips et al. NATURE BIOTECHNOLOGY
- 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
- Broadening the phenotype of TARDBP mutations: the TARDBP Ala382Thr mutation and Parkinson’s disease in Sardinia
- (2011) Marialuisa Quadri et al. NEUROGENETICS
- An autopsy case of SOD1-related ALS with TDP-43 positive inclusions
- (2011) Y. Okamoto et al. NEUROLOGY
- 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
- 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
- Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset
- (2011) Christine Vande Velde et al. PLoS One
- Intermolecular transmission of superoxide dismutase 1 misfolding in living cells
- (2011) L. I. Grad et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells
- (2011) Christian Münch et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Elevated CSF TDP-43 levels in amyotrophic lateral sclerosis: Specificity, sensitivity, and a possible prognostic value
- (2010) Yu-Ichi Noto et al. Amyotrophic Lateral Sclerosis
- Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration
- (2010) Agnes L. Nishimura et al. BRAIN
- Aberrant RNA processing events in neurological disorders
- (2010) Karen Anthony et al. BRAIN RESEARCH
- Research Advances in Amyotrophic Lateral Sclerosis, 2009 to 2010
- (2010) Rebecca Traub et al. Current Neurology and Neuroscience Reports
- The neurobiology of amyotrophic lateral sclerosis
- (2010) André Bento-Abreu et al. EUROPEAN JOURNAL OF NEUROSCIENCE
- Neurotoxic effects of TDP-43 overexpression in C. elegans
- (2010) Peter E.A. Ash et al. HUMAN MOLECULAR GENETICS
- Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice
- (2010) L. Wang et al. HUMAN MOLECULAR GENETICS
- Blocking the mitochondrial apoptotic pathway preserves motor neuron viability and function in a mouse model of amyotrophic lateral sclerosis
- (2010) Nichole A. Reyes et al. JOURNAL OF CLINICAL INVESTIGATION
- Neuronal over-expression of chromogranin A accelerates disease onset in a mouse model of ALS
- (2010) Samer Abou Ezzi et al. JOURNAL OF NEUROCHEMISTRY
- Endoplasmic Reticulum Stress in Motor Neurons of the Spinal Cord in Sporadic Amyotrophic Lateral Sclerosis
- (2010) Shoichi Sasaki JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
- Induction of Protective Immunity by Vaccination With Wild-Type Apo Superoxide Dismutase 1 in Mutant SOD1 Transgenic Mice
- (2010) Shigeko Takeuchi et al. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
- Phosphorylation Promotes Neurotoxicity in a Caenorhabditis elegans Model of TDP-43 Proteinopathy
- (2010) N. F. Liachko et al. JOURNAL OF NEUROSCIENCE
- 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
- Mutations of optineurin in amyotrophic lateral sclerosis
- (2010) Hirofumi Maruyama et al. NATURE
- Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS
- (2010) Daryl A Bosco et al. NATURE NEUROSCIENCE
- Progressive motor weakness in transgenic mice expressing human TDP-43
- (2010) Nancy R. Stallings et al. NEUROBIOLOGY OF DISEASE
- Derlin-1 overexpression ameliorates mutant SOD1-induced endoplasmic reticulum stress by reducing mutant SOD1 accumulation
- (2010) Akira Mori et al. NEUROCHEMISTRY INTERNATIONAL
- Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALS
- (2010) Adrian Israelson et al. NEURON
- Superoxide Dismutase 1 and tgSOD1G93A Mouse Spinal Cord Seed Fibrils, Suggesting a Propagative Cell Death Mechanism in Amyotrophic Lateral Sclerosis
- (2010) Ruth Chia 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
- 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
- TARDBPmutations in motoneuron disease with frontotemporal lobar degeneration
- (2009) Lina Benajiba et al. ANNALS OF NEUROLOGY
- Lack of evidence of monomer/misfolded superoxide dismutase-1 in sporadic amyotrophic lateral sclerosis
- (2009) Hsueh-Ning Liu et al. ANNALS OF NEUROLOGY
- Rethinking ALS: The FUS about TDP-43
- (2009) Clotilde Lagier-Tourenne et al. CELL
- TDP-43 Dimerizes in Human Cells in Culture
- (2009) Yuki Shiina et al. CELLULAR AND MOLECULAR NEUROBIOLOGY
- Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6
- (2009) Fabienne C Fiesel et al. EMBO JOURNAL
- Extracellular mutant SOD1 induces microglial-mediated motoneuron injury
- (2009) Weihua Zhao et al. GLIA
- Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43
- (2009) Takashi Nonaka 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
- Mutation withinTARDBPleads to Frontotemporal Dementia without motor neuron disease
- (2009) B. Borroni et al. HUMAN MUTATION
- TDP-43 Depletion Induces Neuronal Cell Damage through Dysregulation of Rho Family GTPases
- (2009) Yohei Iguchi et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Characterization of Alternative Isoforms and Inclusion Body of the TAR DNA-binding Protein-43
- (2009) Yoshinori Nishimoto 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
- Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond
- (2009) Hristelina Ilieva et al. JOURNAL OF CELL BIOLOGY
- Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates
- (2009) Pei-Hsien Ren et al. NATURE CELL BIOLOGY
- A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice
- (2009) Smita Saxena et al. NATURE NEUROSCIENCE
- Prion-like mechanisms in neurodegenerative diseases
- (2009) Bess Frost et al. NATURE REVIEWS NEUROSCIENCE
- Involvement of CHOP, an ER-stress apoptotic mediator, in both human sporadic ALS and ALS model mice
- (2009) Yasushi Ito et al. NEUROBIOLOGY OF DISEASE
- The Transcellular Spread of Cytosolic Amyloids, Prions, and Prionoids
- (2009) Adriano Aguzzi et al. NEURON
- Axonal ligation induces transient redistribution of TDP-43 in brainstem motor neurons
- (2009) T. Sato et al. NEUROSCIENCE
- Inclusion formation and neuronal cell death through neuron-to-neuron transmission of -synuclein
- (2009) P. Desplats et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration
- (2009) I. Wegorzewska et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Increased TDP-43 protein in cerebrospinal fluid of patients with amyotrophic lateral sclerosis
- (2008) Takashi Kasai et al. ACTA NEUROPATHOLOGICA
- 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
- TDP-43mutation in familial amyotrophic lateral sclerosis
- (2008) Akio Yokoseki et al. ANNALS OF NEUROLOGY
- TDP-43 in Cerebrospinal Fluid of Patients With Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
- (2008) Petra Steinacker et al. ARCHIVES OF NEUROLOGY
- Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: Implications for TDP-43 in the physiological response to neuronal injury
- (2008) Katie Moisse et al. BRAIN RESEARCH
- Human Embryonic Stem Cell-Derived Motor Neurons Are Sensitive to the Toxic Effect of Glial Cells Carrying an ALS-Causing Mutation
- (2008) Francesco Paolo Di Giorgio et al. Cell Stem Cell
- The endoplasmic reticulum-Golgi pathway is a target for translocation and aggregation of mutant superoxide dismutase linked to ALS
- (2008) Makoto Urushitani et al. FASEB JOURNAL
- ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1
- (2008) H. Nishitoh et al. GENES & DEVELOPMENT
- Conformational Diversity of Wild-type Tau Fibrils Specified by Templated Conformation Change
- (2008) Bess Frost et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- 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
- TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia
- (2008) C C Weihl et al. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
- Colocalization of Transactivation-Responsive DNA-Binding Protein 43 and Huntingtin in Inclusions of Huntington Disease
- (2008) Claudia Schwab et al. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
- Nuclear TAR DNA Binding Protein 43 Expression in Spinal Cord Neurons Correlates With the Clinical Course in Amyotrophic Lateral Sclerosis
- (2008) Hisae Sumi et al. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
- 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
- Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis
- (2008) Koji Yamanaka et al. NATURE NEUROSCIENCE
- Neuronal inclusion protein TDP-43 has no primary genetic role in FTD and ALS
- (2008) Ilse Gijselinck et al. NEUROBIOLOGY OF AGING
- Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis
- (2008) Julie D. Atkin et al. NEUROBIOLOGY OF DISEASE
- Pallidonigral TDP-43 pathology in Perry syndrome
- (2008) Christian Wider et al. PARKINSONISM & RELATED DISORDERS
- TDP-43 Is Not a Common Cause of Sporadic Amyotrophic Lateral Sclerosis
- (2008) Rita J. Guerreiro et al. PLoS One
- TDP-43 regulates retinoblastoma protein phosphorylation through the repression of cyclin-dependent kinase 6 expression
- (2008) Y. M. Ayala et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity
- (2008) B. S. Johnson et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- 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
- Restricted expression of mutant SOD1 in spinal motor neurons and interneurons induces motor neuron pathology
- (2007) Lijun Wang et al. NEUROBIOLOGY OF DISEASE
Create your own webinar
Interested in hosting your own webinar? Check the schedule and propose your idea to the Peeref Content Team.
Create NowBecome a Peeref-certified reviewer
The Peeref Institute provides free reviewer training that teaches the core competencies of the academic peer review process.
Get Started