Associated Papers
From Dish to Trial: Building Translational Models of ALS.
Salamotas I et al.
Cells, 2026
Missense mutations in intrinsically disordered protein regions link pathogenicity and phase separation.
Kipp OL et al.
The Journal of Biological Chemistry, 2025
Integrated profiling of iPSC-derived motor neurons carrying C9orf72, FUS, TARDBP, or SOD1 mutations.
Ma GM et al.
Stem Cell Reports, 2025
Genetic and structural aspects of amyloid diseases.
Rosenberg GM et al.
Science Translational Medicine, 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
Perspectives in Amyotrophic Lateral Sclerosis: Biomarkers, Omics, and Gene Therapy Informing Disease and Treatment.
Bono N et al.
International Journal of Molecular Sciences, 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
Modelling Population Genetic Screening in Rare Neurodegenerative Diseases.
Spargo TP et al.
Biomedicines, 2025
Amyotrophic lateral sclerosis caused by FUS mutations: advances with broad implications.
Moens TG et al.
The Lancet. Neurology, 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
FUS-related amyotrophic lateral sclerosis-frontotemporal dementia and links to the DNA damage response: a systematic review.
Almalki S et al.
Frontiers in Molecular Neuroscience, 2025
Oxidative stress-induced stress granules: a central link to protein aggregation in neurodegenerative diseases.
Younas N et al.
Frontiers in Neuroscience, 2025
Screens in aging-relevant human ALS-motor neurons identify MAP4Ks as therapeutic targets for the disease.
Liu ML et al.
Cell Death & Disease, 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
Amyotrophic Lateral Sclerosis, FUS and Protein Synthesis Defects.
Assoni AF et al.
Stem Cell Reviews and Reports, 2023
Mutated FUS in familial amyotrophic lateral sclerosis involves multiple hnRNPs in the formation of neuronal cytoplasmic inclusions.
Honda H et al.
Journal of Neuropathology and Experimental Neurology, 2023
Genetic Interaction of tRNA-Dependent Mistranslation with Fused in Sarcoma Protein Aggregates.
Lant JT et al.
Genes, 2023
PIKFYVE inhibition mitigates disease in models of diverse forms of ALS.
Hung ST et al.
Cell, 2023
FUS Alters circRNA Metabolism in Human Motor Neurons Carrying the ALS-Linked P525L Mutation.
Colantoni A et al.
International Journal of Molecular Sciences, 2023
FUS-ALS hiPSC-derived astrocytes impair human motor units through both gain-of-toxicity and loss-of-support mechanisms.
Stoklund Dittlau K et al.
Molecular Neurodegeneration, 2023
Study of Ubiquitin Pathway Genes in a French Population with Amyotrophic Lateral Sclerosis: Focus on HECW1 Encoding the E3 Ligase NEDL1.
Haouari S et al.
International Journal of Molecular Sciences, 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
Proteostasis impairment and ALS.
Lambert-Smith IA et al.
Progress in Biophysics and Molecular Biology, 2022
FUS-P525L Juvenile Amyotrophic Lateral Sclerosis and Intellectual Disability: Evidence for Association and Oligogenic Inheritance.
Goldstein O et al.
Neurology. Genetics, 2022
Upregulation of β-catenin due to loss of miR-139 contributes to motor neuron death in amyotrophic lateral sclerosis.
Hawkins S et al.
Stem Cell Reports, 2022
A streamlined CRISPR workflow to introduce mutations and generate isogenic iPSCs for modeling amyotrophic lateral sclerosis.
Deneault E et al.
Methods (san Diego, Calif.), 2022
Nearly 30 Years of Animal Models to Study Amyotrophic Lateral Sclerosis: A Historical Overview and Future Perspectives.
Bonifacino T et al.
International Journal of Molecular Sciences, 2021
Insights into amyotrophic lateral sclerosis linked Pro525Arg mutation in the fused in sarcoma protein through in silico analysis and molecular dynamics simulation.
Chatterjee S et al.
Journal of Biomolecular Structure & Dynamics, 2021
Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway.
Lin YC et al.
Nature Neuroscience, 2021
Emerging hiPSC Models for Drug Discovery in Neurodegenerative Diseases.
Trudler D et al.
International Journal of Molecular Sciences, 2021
Destination Amyotrophic Lateral Sclerosis.
Keon M et al.
Frontiers in Neurology, 2021
Stress granule subtypes: an emerging link to neurodegeneration.
Advani VM et al.
Cellular and Molecular Life Sciences : Cmls, 2020
A de novo c.1509dupA:p.R503fs mutation of FUS: report of a girl with sporadic juvenile amyotrophic lateral sclerosis.
Chen L et al.
Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, 2020
Fused in Sarcoma (FUS) in DNA Repair: Tango with Poly(ADP-ribose) Polymerase 1 and Compartmentalisation of Damaged DNA.
Sukhanova MV et al.
International Journal of Molecular Sciences, 2020
Modeling ALS using iPSCs: is it possible to reproduce the phenotypic variations observed in patients in vitro?
Vasques JF et al.
Regenerative Medicine, 2020
CRISPR/Cas9-Mediated Gene Correction to Understand ALS.
Yun Y et al.
International Journal of Molecular Sciences, 2020
Connecting RNA-Modifying Similarities of TDP-43, FUS, and SOD1 with MicroRNA Dysregulation Amidst A Renewed Network Perspective of Amyotrophic Lateral Sclerosis Proteinopathy.
Pham J et al.
International Journal of Molecular Sciences, 2020
Examining the relationship between astrocyte dysfunction and neurodegeneration in ALS using hiPSCs.
Halpern M et al.
Neurobiology of Disease, 2019
The RNA-binding protein FUS/TLS undergoes calcium-mediated nuclear egress during excitotoxic stress and is required for GRIA2 mRNA processing.
Tischbein M et al.
The Journal of Biological Chemistry, 2019
Fused in Sarcoma: Properties, Self-Assembly and Correlation with Neurodegenerative Diseases.
Chen C et al.
Molecules (basel, Switzerland), 2019
Astrocytes in Motor Neuron Diseases.
Valori CF et al.
Advances in Experimental Medicine and Biology, 2019
Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains.
Guo L et al.
Cell, 2018
Properties of LINE-1 proteins and repeat element expression in the context of amyotrophic lateral sclerosis.
Pereira GC et al.
Mobile Dna, 2018
Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.
Gao FB et al.
The Embo Journal, 2017