Associated Papers
TDP-43 proteinopathies and neurodegeneration: insights from Caenorhabditis elegans models.
Pir GJ et al.
The Febs Journal, 2026
TRAF6 regulates ubiquitination-independent TDP-43 condensation and related neurodegeneration.
Guo S et al.
Molecular Psychiatry, 2025
Pathological forms of TDP-43 in amyotrophic lateral sclerosis (ALS) promote aberrant telomere elongation.
Konopka A et al.
Biochimica Et Biophysica Acta. Molecular Basis of Disease, 2025
Cryptic Splicing of GAP43 mRNA is a Novel Hallmark of TDP-43-Associated ALS and AD.
Yang M et al.
Advanced Science (weinheim, Baden-wurttemberg, Germany), 2025
Biobank-scale genetic characterization of Alzheimer's disease and related dementias across diverse ancestries.
Khani M et al.
Nature Communications, 2025
Deep learning analyses of splicing variants identify the link of PCP4 with amyotrophic lateral sclerosis.
Tang X et al.
Brain : a Journal of Neurology, 2025
Myopathic aggregation-prone variants in the TDP-43 prion-like domain: genetics paving the way.
Ervilha Pereira P et al.
Brain : a Journal of Neurology, 2025
Exploring Protein Misfolding in Amyotrophic Lateral Sclerosis: Structural and Functional Insights.
Ivantsik O et al.
Biomedicines, 2025
Molecular Mechanisms of Protein Aggregation in ALS-FTD: Focus on TDP-43 and Cellular Protective Responses.
Verde EM et al.
Cells, 2025
Amyotrophic lateral sclerosis caused by TARDBP mutations: from genetics to TDP-43 proteinopathy.
Balendra R et al.
The Lancet. Neurology, 2025
Endogenous TDP-43 mislocalization in a novel knock-in mouse model reveals DNA repair impairment, inflammation, and neuronal senescence.
Mitra J et al.
Acta Neuropathologica Communications, 2025
Decoding TDP-43: the molecular chameleon of neurodegenerative diseases.
Zeng J et al.
Acta Neuropathologica Communications, 2024
Lycorine protects motor neurons against TDP-43 proteinopathy-induced degeneration in cross-species models with amyotrophic lateral sclerosis.
Wen J et al.
Pharmacological Research, 2024
Single Acetylation-mimetic Mutation in TDP-43 Nuclear Localization Signal Disrupts Importin α1/β Signaling.
Ko YH et al.
Journal of Molecular Biology, 2024
N-Terminal Fragments of TDP-43-In Vitro Analysis and Implication in the Pathophysiology of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration.
Chami AA et al.
Genes, 2024
Post-Translational Variants of Major Proteins in Amyotrophic Lateral Sclerosis Provide New Insights into the Pathophysiology of the Disease.
Bedja-Iacona L et al.
International Journal of Molecular Sciences, 2024
ALS Identified: two-year findings from a sponsored ALS genetic testing program.
Goutman SA et al.
Annals of Clinical and Translational Neurology, 2024
In vivo diagnosis of TDP-43 proteinopathies: in search of biomarkers of clinical use.
López-Carbonero JI et al.
Translational Neurodegeneration, 2024
Plasma extracellular vesicle tau and TDP-43 as diagnostic biomarkers in FTD and ALS.
Chatterjee M et al.
Nature Medicine, 2024
Skeletal myotubes expressing ALS mutant SOD1 induce pathogenic changes, impair mitochondrial axonal transport, and trigger motoneuron death.
Martínez P et al.
Biorxiv : the Preprint Server for Biology, 2024
Endogenous TDP-43 mislocalization in a novel knock-in mouse model reveals DNA repair impairment, inflammation, and neuronal senescence.
Mitra J et al.
Research Square, 2024
A complex network of interdomain interactions underlies the conformational ensemble of monomeric TDP-43 and modulates its phase behavior.
Mohanty P et al.
Protein Science : a Publication of the Protein Society, 2024
Nuclear-import receptors as gatekeepers of pathological phase transitions in ALS/FTD.
Khalil B et al.
Molecular Neurodegeneration, 2024
Astrocyte-Neuron Interactions Contributing to Amyotrophic Lateral Sclerosis Progression.
Jensen BK
Advances in Neurobiology, 2024
Structural Integrity of Nucleolin Is Required to Suppress TDP-43-Mediated Cytotoxicity in Yeast and Human Cell Models.
Peggion C et al.
International Journal of Molecular Sciences, 2023
Variability in Clinical Phenotype in TARDBP Mutations: Amyotrophic Lateral Sclerosis Case Description and Literature Review.
Lombardi M et al.
Genes, 2023
TDP-43 protein interactome informs about perturbed canonical pathways and may help develop personalized medicine approaches for patients with TDP-43 pathology.
Helmold BR et al.
Drug Discovery Today, 2023
TDP-43 Epigenetic Facets and Their Neurodegenerative Implications.
Gimenez J et al.
International Journal of Molecular Sciences, 2023
Immunotherapy targeting the C-terminal domain of TDP-43 decreases neuropathology and confers neuroprotection in mouse models of ALS/FTD.
Afroz T et al.
Neurobiology of Disease, 2023
Shapeshifter TDP-43: Molecular mechanism of structural polymorphism, aggregation, phase separation and their modulators.
Doke AA et al.
Biophysical Chemistry, 2023
Loss of TDP-43 function underlies hippocampal and cortical synaptic deficits in TDP-43 proteinopathies.
Ni J et al.
Molecular Psychiatry, 2023
Characterising the Genetic Landscape of Amyotrophic Lateral Sclerosis: A Catalogue and Assessment of Over 1,000 Published Genetic Variants.
McCann EP et al.
Journal of Neuromuscular Diseases, 2023
Mature iPSC-derived astrocytes of an ALS/FTD patient carrying the TDP43A90V mutation display a mild reactive state and release polyP toxic to motoneurons.
Rojas F et al.
Frontiers in Cell and Developmental Biology, 2023
Nuclear import receptors are recruited by FG-nucleoporins to rescue hallmarks of TDP-43 proteinopathy.
Khalil B et al.
Molecular Neurodegeneration, 2022
Importin α/β and the tug of war to keep TDP-43 in solution: quo vadis?
Doll SG et al.
Bioessays : News and Reviews in Molecular, Cellular and Developmental Biology, 2022
Identification of TARDBP Gly298Ser as a founder mutation for amyotrophic lateral sclerosis in Southern China.
Xu F et al.
Bmc Medical Genomics, 2022
Altered TDP-43 Structure and Function: Key Insights into Aberrant RNA, Mitochondrial, and Cellular and Systemic Metabolism in Amyotrophic Lateral Sclerosis.
Jiang L et al.
Metabolites, 2022
The Role of TDP-43 in Neurodegenerative Disease.
Liao YZ et al.
Molecular Neurobiology, 2022
Recognition of the TDP-43 nuclear localization signal by importin α1/β.
Doll SG et al.
Cell Reports, 2022
Excessive release of inorganic polyphosphate by ALS/FTD astrocytes causes non-cell-autonomous toxicity to motoneurons.
Arredondo C et al.
Neuron, 2022
Revealing the Mutational Spectrum in Southern Africans With Amyotrophic Lateral Sclerosis.
Nel M et al.
Neurology. Genetics, 2022
Expanding the TDP-43 Proteinopathy Pathway From Neurons to Muscle: Physiological and Pathophysiological Functions.
Versluys L et al.
Frontiers in Neuroscience, 2022
Sequence Determinants of TDP-43 Ribonucleoprotein Condensate Formation and Axonal Transport in Neurons.
Vishal SS et al.
Frontiers in Cell and Developmental Biology, 2022
Functional implication of ubiquitinating and deubiquitinating mechanisms in TDP-43 proteinopathies.
Tran NN et al.
Frontiers in Cell and Developmental Biology, 2022
TDP-43 Pathology in Alzheimer's Disease.
Meneses A et al.
Molecular Neurodegeneration, 2021
Niclosamide affects intracellular TDP-43 distribution in motor neurons, activates mitophagy, and attenuates morphological changes under stress.
Kato Y et al.
Journal of Bioscience and Bioengineering, 2021