Associated Papers
Targeting lipid droplets in FUS-linked amyotrophic lateral sclerosis mitigates neuronal and astrocytic lipotoxicity.
Marcadet L et al.
Brain : a Journal of Neurology, 2026
Defining RNA oligonucleotides that reverse deleterious phase transitions of RNA-binding proteins with prion-like domains.
Guo L et al.
Molecular Cell, 2026
Axonal Eif5a hypusination controls local translation and mitigates defects in FUS-ALS.
Piol D et al.
Nature Neuroscience, 2026
Genetic Spectrum and Phenotypic Variability in Chinese Patients with Multisystem Proteinopathy and Related Disorders.
Xia X et al.
Degenerative Neurological and Neuromuscular Disease, 2026
Investigation of mitochondrial phenotypes in motor neurons derived by direct conversion of fibroblasts from familial ALS subjects.
Woo E et al.
Cell Death & Disease, 2025
Integrated profiling of iPSC-derived motor neurons carrying C9orf72, FUS, TARDBP, or SOD1 mutations.
Ma GM et al.
Stem Cell Reports, 2025
Protein Structure-based FUS Mutational Subtypes Are Associated With Protein Mislocalization in Amyotrophic Lateral Sclerosis Patients.
Yang W et al.
Molecular Neurobiology, 2025
Integrative Transcriptomic and Network-Based Analysis of Neuromuscular Diseases.
García-Criado F et al.
International Journal of Molecular Sciences, 2025
Transcriptomic Alteration in FUS-ALS Points Towards Apoptosis-Rather than Ferroptosis-Related Cell Death Pathway.
Dash BP et al.
Cells, 2025
The Impact of Splicing Dysregulation on Neuromuscular Disorders and Current Neuromuscular Genetic Therapies.
Wu J et al.
Journal of Neurochemistry, 2025
DNA nanoflower Oligo-PROTAC for targeted degradation of FUS to treat neurodegenerative diseases.
Ge R et al.
Nature Communications, 2025
Single-cell RNA-sequencing reveals early mitochondrial dysfunction unique to motor neurons shared across FUS- and TARDBP-ALS.
Schweingruber C et al.
Nature Communications, 2025
Exploring Protein Misfolding in Amyotrophic Lateral Sclerosis: Structural and Functional Insights.
Ivantsik O et al.
Biomedicines, 2025
TDP-43 seeding activity in the olfactory mucosa of patients with amyotrophic lateral sclerosis.
Vizziello M et al.
Molecular Neurodegeneration, 2025
Modelling Population Genetic Screening in Rare Neurodegenerative Diseases.
Spargo TP et al.
Biomedicines, 2025
A population based study to analyse amyotrophic lateral sclerosis as a multi-step process.
D'Amico A et al.
Scientific Reports, 2025
Conformational switches in human RNA binding proteins involved in neurodegeneration.
Chatterjee S et al.
Biochimica Et Biophysica Acta. General Subjects, 2025
Investigation of mitochondrial phenotypes in motor neurons derived by direct conversion of fibroblasts from familial ALS subjects.
Woo E et al.
Biorxiv : the Preprint Server for Biology, 2025
Targeting common disease pathomechanisms to treat amyotrophic lateral sclerosis.
Faller KME et al.
Nature Reviews. Neurology, 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
Case Report: A case of ALS type 6 associated with a FUS gene variant and right limb muscle weakness and atrophy as the initial symptom.
Zhan X et al.
Frontiers in Genetics, 2025
Localized molecular chaperone synthesis maintains neuronal dendrite proteostasis.
Alecki C et al.
Nature Communications, 2024
Axonopathy Underlying Amyotrophic Lateral Sclerosis: Unraveling Complex Pathways and Therapeutic Insights.
Luan T et al.
Neuroscience Bulletin, 2024
Decoding Missense Variants by Incorporating Phase Separation via Machine Learning.
Feng M et al.
Nature Communications, 2024
Poly-GR repeats associated with ALS/FTD gene C9ORF72 impair translation elongation and induce a ribotoxic stress response in neurons.
Dong D et al.
Science Signaling, 2024
Clinical and genetic characteristics of 1672 cases of amyotrophic lateral sclerosis in China: a single-center retrospective study.
Shen D et al.
Journal of Neurology, 2024
ALS Identified: two-year findings from a sponsored ALS genetic testing program.
Goutman SA et al.
Annals of Clinical and Translational Neurology, 2024
Divergent Molecular Pathways for Toxicity of Selected Mutant C9ORF72-derived Dipeptide Repeats.
Okekenwa S et al.
Biorxiv : the Preprint Server for Biology, 2024
High-content analysis of proteostasis capacity in cellular models of amyotrophic lateral sclerosis (ALS).
Lambert-Smith IA et al.
Scientific Reports, 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
Updates on Disease Mechanisms and Therapeutics for Amyotrophic Lateral Sclerosis.
Nguyen L
Cells, 2024
The implications of physiological biomolecular condensates in amyotrophic lateral sclerosis.
Fakim H et al.
Seminars in Cell & Developmental Biology, 2024
Cytoplasmic retention of the DNA/RNA-binding protein FUS ameliorates organ fibrosis in mice.
Chiusa M et al.
The Journal of Clinical Investigation, 2024
Molecular hallmarks of ageing in amyotrophic lateral sclerosis.
Jagaraj CJ et al.
Cellular and Molecular Life Sciences : Cmls, 2024
PP2A and GSK3 act as modifiers of FUS-ALS by modulating mitochondrial transport.
Tziortzouda P et al.
Acta Neuropathologica, 2024
FUS gene mutation in amyotrophic lateral sclerosis: a new case report and systematic review.
Xiao X et al.
Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 2024
Screens in aging-relevant human ALS-motor neurons identify MAP4Ks as therapeutic targets for the disease.
Liu ML et al.
Cell Death & Disease, 2024
IFNγ protects motor neurons from oxidative stress via enhanced global protein synthesis in FUS-associated amyotrophic lateral sclerosis.
Assoni AF et al.
Brain Pathology (zurich, Switzerland), 2024
Animal Models of FUS-Proteinopathy: A Systematic Review.
Rezvykh A et al.
Biochemistry. Biokhimiia, 2024
Astrocyte-Neuron Interactions Contributing to Amyotrophic Lateral Sclerosis Progression.
Jensen BK
Advances in Neurobiology, 2024
Localized molecular chaperone synthesis maintains neuronal dendrite proteostasis.
Ugalde MV et al.
Research Square, 2023
Neuronal dysfunction caused by FUSR521G promotes ALS-associated phenotypes that are attenuated by NF-κB inhibition.
Pelaez MC et al.
Acta Neuropathologica Communications, 2023
The Key Role of Astrocytes in Amyotrophic Lateral Sclerosis and Their Commitment to Glutamate Excitotoxicity.
Provenzano F et al.
International Journal of Molecular Sciences, 2023
Pathological insights from amyotrophic lateral sclerosis animal models: comparisons, limitations, and challenges.
Zhu L et al.
Translational Neurodegeneration, 2023
Defining RNA oligonucleotides that reverse deleterious phase transitions of RNA-binding proteins with prion-like domains.
Guo L et al.
Biorxiv : the Preprint Server for Biology, 2023