Two neuronal models of TDP-43 proteinopathy display reduced axonal translation, increased oxidative stress, and defective exocytosis (original) (raw)
Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations
Edward Chaum, Nael H Alami
Neuron, 2014
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ALS-Linked Mutations Enlarge TDP-43-Enriched Neuronal RNA Granules in the Dendritic Arbor
A. Ebata
Journal of Neuroscience, 2014
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Cytoplasmic Expression of the ALS/FTD-Related Protein TDP-43 Decreases Global Translation Both in vitro and in vivo
Matias Blaustein
Frontiers in Cellular Neuroscience, 2020
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Altered TDP-43 Structure and Function: Key Insights into Aberrant RNA, Mitochondrial, and Cellular and Systemic Metabolism in Amyotrophic Lateral Sclerosis
Leanne Jiang
Metabolites
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Loss of Tdp-43 disrupts the axonal transcriptome of motoneurons accompanied by impaired axonal translation and mitochondria function
michael sendtner
Acta Neuropathologica Communications, 2020
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Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy
Matthew Stopford, Robin Highley, Johnathan Cooper-Knock, Janine Kirby
Acta Neuropathologica, 2015
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Increased cytoplasmicTARDBPmRNA in affected spinal motor neurons in ALS caused by abnormal autoregulation of TDP-43
Osamu Onodera
Nucleic Acids Research, 2016
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Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs
Magdalini Polymenidou
Nature Neuroscience, 2012
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Loss of nuclear TDP‐43 in amyotrophic lateral sclerosis (ALS) causes altered expression of splicing machinery and widespread dysregulation of RNA splicing in motor neurones
Pamela Shaw
Neuropathology and Applied Neurobiology, 2014
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TDP-43 enhances translation of specific mRNAs linked to neurodegenerative disease
Giorgio Gonnella
Nucleic Acids Research, 2018
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Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses
jiayu zhang
Nature communications, 2016
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RNA targets of TDP-43 identified by UV-CLIP are deregulated in ALS
Lorne Zinman, David Sheps
Molecular and cellular neurosciences, 2011
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Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death
Lorne Zinman
Acta neuropathologica, 2015
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Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis
Leonardo Aliaga
Proceedings of the National Academy of Sciences, 2014
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Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALS
Osamu Onodera
PloS one, 2012
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{"__content__"=>"Robustness and Vulnerability of the Autoregulatory System That Maintains Nuclear TDP-43 Levels: A Trade-off Hypothesis for ALS Pathology Based on Data.", "i"=>{"__content__"=>"in Silico"}}
Osamu Onodera
Frontiers in neuroscience, 2018
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A Computational Approach to Investigate TDP-43 RNA-Recognition Motif 2 C-Terminal Fragments Aggregation in Amyotrophic Lateral Sclerosis
Elsa Zacco
Biomolecules, 2021
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TDP-43: gumming up neurons through protein-protein and protein-RNA interactions
Emanuele Buratti
2012
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Identification of RNA bound to the TDP-43 ribonucleoprotein complex in the adult mouse brain
Ajay Panwar
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2012
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Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders
Christine Van Broeckhoven
Human Molecular Genetics, 2013
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Multi-phaseted problems of TDP-43 in selective neuronal vulnerability in ALS
Kazuhide Asakawa
Cellular and Molecular Life Sciences
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Global Analysis of TDP-43 Interacting Proteins Reveals Strong Association with RNA Splicing and Translation Machinery
Raghu Chitta
Journal of Proteome Research, 2010
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TDP-43 and FUS RNA-binding Proteins Bind Distinct Sets of Cytoplasmic Messenger RNAs and Differently Regulate Their Post-transcriptional Fate in Motoneuron-like Cells
Emanuele Buratti
Journal of Biological Chemistry, 2012
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RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43
Bryan Hurtle
Neuron, 2019
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MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis
Lorne Zinman, Jean-Pierre Julien
Brain : a journal of neurology, 2015
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Cell environment shapes TDP-43 function with implications in neuronal and muscle disease
Emanuele Buratti
Communications Biology
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TDP-43 proteinopathies: a new wave of neurodegenerative diseases
Parvathi Menon
Journal of Neurology, Neurosurgery & Psychiatry
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The role of TDP-43 in the pathogenesis of ALS and FTLD
Emanuele Buratti
2013
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Advances in Neurobiology 20 RNA Metabolism in Neurodegenerative Diseases
Antia Alvarez Pazo
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TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration
Magdalini Polymenidou
Human Molecular Genetics, 2010
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Cell environment shapes TDP-43 function: implications in neuronal and muscle disease
Emanuele Buratti
2021
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