Associated Papers
TARDBP (TDP-43) Knock-in Zebrafish Display a Late-Onset Motor Phenotype and Loss of Large Spinal Cord Motor Neurons.
Harji ZA et al.
Annals of Neurology, 2026
TDP-43 proteinopathies and neurodegeneration: insights from Caenorhabditis elegans models.
Pir GJ et al.
The Febs Journal, 2026
Human TDP-43 overexpression in zebrafish motor neurons triggers MND-like phenotypes through gain-of-function mechanism.
Hogan AL et al.
Acta Neuropathologica Communications, 2025
Genetic spectrum among 2009 Iranian individuals with neuromuscular disorders using next generation sequencing and multiple ligation dependent probe amplification methods.
Molaei N et al.
Scientific Reports, 2025
Antibody targeting TDP-43 mitigates pathogenic pathways induced by the cerebrospinal fluid of ALS.
Poulin-Brière A et al.
Neurotherapeutics : the Journal of the American Society for Experimental Neurotherapeutics, 2025
Sephin1 reduces TDP-43 cytoplasmic mislocalization and improves motor neuron survival in ALS models.
Abgueguen E et al.
Life Science Alliance, 2025
Transcriptome-based screening in TARDBP/TDP-43 knock-in motor neurons identifies the NEDD8-activating enzyme inhibitor MLN4924.
Lépine S et al.
Scientific Reports, 2025
TDP-43 mutants with different aggregation properties exhibit distinct toxicity, axonal transport, and secretion for disease progression in a mouse ALS/FTLD model.
Mori H et al.
Neurobiology of Disease, 2025
TARDBP variants in Taiwanese ALS patients: Genetic spectrum, clinical features, and founder effects.
Jih KY et al.
Journal of the Neurological Sciences, 2025
Human TDP-43 overexpression in zebrafish motor neurons triggers MND-like phenotypes through gain-of-function mechanism.
Hogan AL et al.
Biorxiv : the Preprint Server for Biology, 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
Challenges of modelling TDP-43 pathology in mice.
Armas JMB et al.
Mammalian Genome : Official Journal of the International Mammalian Genome Society, 2025
Exploring Protein Misfolding in Amyotrophic Lateral Sclerosis: Structural and Functional Insights.
Ivantsik O et al.
Biomedicines, 2025
Molecular mechanisms and consequences of TDP-43 phosphorylation in neurodegeneration.
Kellett EA et al.
Molecular Neurodegeneration, 2025
Amyotrophic lateral sclerosis and frontotemporal dementia mutation reduces endothelial TDP-43 and causes blood-brain barrier defects.
Cheemala A et al.
Science Advances, 2025
The Regulation of TDP-43 Structure and Phase Transitions: A Review.
Liu Y et al.
The Protein Journal, 2025
Comprehensive mapping of mutations in TDP-43 and α-Synuclein that affect stability and binding.
Alamri SH et al.
Journal of Biomolecular Structure & Dynamics, 2025
Therapeutic targeting of the oxidative stress generated by pathological molecular pathways in the neurodegenerative diseases, ALS and Huntington's.
Bajpai A et al.
European Journal of Pharmacology, 2025
A systematic review and functional in-silico analysis of genes and variants associated with amyotrophic lateral sclerosis.
Arreola-Aldape CA et al.
Frontiers in Neuroscience, 2025
TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: From pathomechanisms to therapeutic strategies.
Ho PC et al.
Ageing Research Reviews, 2024
ALS Identified: two-year findings from a sponsored ALS genetic testing program.
Goutman SA et al.
Annals of Clinical and Translational Neurology, 2024
Synaptopathy: presynaptic convergence in frontotemporal dementia and amyotrophic lateral sclerosis.
Clayton EL et al.
Brain : a Journal of Neurology, 2024
Impact of histone deacetylase inhibition and arimoclomol on heat shock protein expression and disease biomarkers in primary culture models of familial ALS.
Fernández Comaduran M et al.
Cell Stress & Chaperones, 2024
Homozygous ALS-linked mutations in TARDBP/TDP-43 lead to hypoactivity and synaptic abnormalities in human iPSC-derived motor neurons.
Lépine S et al.
Iscience, 2024
Molecular hallmarks of ageing in amyotrophic lateral sclerosis.
Jagaraj CJ et al.
Cellular and Molecular Life Sciences : Cmls, 2024
IκB kinase phosphorylates cytoplasmic TDP-43 and promotes its proteasome degradation.
Iguchi Y et al.
The Journal of Cell Biology, 2024
Drug Screening and Validation Targeting TDP-43 Proteinopathy for Amyotrophic Lateral Sclerosis.
Xin J et al.
Aging and Disease, 2024
Protein aggregation and therapeutic strategies in SOD1- and TDP-43- linked ALS.
Tsekrekou M et al.
Frontiers in Molecular Biosciences, 2024
Diving deep: zebrafish models in motor neuron degeneration research.
Garg V et al.
Frontiers in Neuroscience, 2024
Loss of Endothelial TDP-43 Leads to Blood Brain Barrier Defects in Mouse Models of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia.
Cheemala A et al.
Biorxiv : the Preprint Server for Biology, 2023
TDP-43 as a therapeutic target in neurodegenerative diseases: Focusing on motor neuron disease and frontotemporal dementia.
Babazadeh A et al.
Ageing Research Reviews, 2023
Variability in Clinical Phenotype in TARDBP Mutations: Amyotrophic Lateral Sclerosis Case Description and Literature Review.
Lombardi M et al.
Genes, 2023
Dysregulated TDP-43 proteostasis perturbs excitability of spinal motor neurons during brainstem-mediated fictive locomotion in zebrafish.
Asakawa K et al.
Development, Growth & Differentiation, 2023
Pathological insights from amyotrophic lateral sclerosis animal models: comparisons, limitations, and challenges.
Zhu L et al.
Translational Neurodegeneration, 2023
TDP-43 Epigenetic Facets and Their Neurodegenerative Implications.
Gimenez J et al.
International Journal of Molecular Sciences, 2023
Transactive response DNA-binding protein 43 is enriched at the centrosome in human cells.
Bodin A et al.
Brain : a Journal of Neurology, 2023
A Novel Drosophila-based Drug Repurposing Platform Identified Fingolimod As a Potential Therapeutic for TDP-43 Proteinopathy.
Lo Piccolo L et al.
Neurotherapeutics : the Journal of the American Society for Experimental Neurotherapeutics, 2023
In vivo analysis of aggregation propensity of low levels of mislocalized TDP-43 on cytopathological and behavioral phenotype of ALS/FTLD.
Wada H et al.
Neuroscience Research, 2023
TDP-43 forms amyloid filaments with a distinct fold in type A FTLD-TDP.
Arseni D et al.
Nature, 2023
Specific vulnerability of iPSC-derived motor neurons with TDP-43 gene mutation to oxidative stress.
Onda-Ohto A et al.
Molecular Brain, 2023
Disrupting pathologic phase transitions in neurodegeneration.
Hurtle BT et al.
The Journal of Clinical Investigation, 2023
Regulation of RNG105/caprin1 dynamics by pathogenic cytoplasmic FUS and TDP-43 in neuronal RNA granules modulates synaptic loss.
Horio T et al.
Heliyon, 2023
Neurofilament accumulations in amyotrophic lateral sclerosis patients' motor neurons impair axonal initial segment integrity.
Lefebvre-Omar C et al.
Cellular and Molecular Life Sciences : Cmls, 2023