Associated Papers
M6A-dependent RNA condensation underlies FUS autoregulation and can be harnessed for ALS therapy development.
Huang WP et al.
Science Advances, 2025
Modelling Population Genetic Screening in Rare Neurodegenerative Diseases.
Spargo TP et al.
Biomedicines, 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
Regulation of DNA damage response by RNA/DNA-binding proteins: Implications for neurological disorders and aging.
Kodavati M et al.
Ageing Research Reviews, 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
Animal Models of FUS-Proteinopathy: A Systematic Review.
Rezvykh A et al.
Biochemistry. Biokhimiia, 2024
Drosha-dependent microRNAs modulate FUS-mediated neurodegeneration in vivo.
Kour S et al.
Nucleic Acids Research, 2023
Axon guidance genes modulate neurotoxicity of ALS-associated UBQLN2.
Kim SH et al.
Elife, 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
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
Nucleoporins are degraded via upregulation of ESCRT-III/Vps4 complex in Drosophila models of C9-ALS/FTD.
Dubey SK et al.
Cell Reports, 2022
The converging roles of sequestosome-1/p62 in the molecular pathways of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD).
Davidson JM et al.
Neurobiology of Disease, 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
Targeted sequencing panels in Italian ALS patients support different etiologies in the ALS/FTD continuum.
Bartoletti-Stella A et al.
Journal of Neurology, 2021
DDX17 is involved in DNA damage repair and modifies FUS toxicity in an RGG-domain dependent manner.
Fortuna TR et al.
Acta Neuropathologica, 2021
Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway.
Lin YC et al.
Nature Neuroscience, 2021
Amyotrophic Lateral Sclerosis Genes in Drosophila melanogaster.
Layalle S et al.
International Journal of Molecular Sciences, 2021
Destination Amyotrophic Lateral Sclerosis.
Keon M et al.
Frontiers in Neurology, 2021
Inactivation of Hippo and cJun-N-terminal Kinase (JNK) signaling mitigate FUS mediated neurodegeneration in vivo.
Gogia N et al.
Neurobiology of Disease, 2020
Amyotrophic Lateral Sclerosis Modifiers in Drosophila Reveal the Phospholipase D Pathway as a Potential Therapeutic Target.
Kankel MW et al.
Genetics, 2020
Karyopherins and condensates.
Springhower CE et al.
Current Opinion in Cell Biology, 2020
CRISPR/Cas9-Mediated Gene Correction to Understand ALS.
Yun Y et al.
International Journal of Molecular Sciences, 2020
Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization.
Casci I et al.
Nature Communications, 2019
The role of mitochondria in amyotrophic lateral sclerosis.
Smith EF et al.
Neuroscience Letters, 2019
Fused in Sarcoma: Properties, Self-Assembly and Correlation with Neurodegenerative Diseases.
Chen C et al.
Molecules (basel, Switzerland), 2019
ALS-linked FUS mutations confer loss and gain of function in the nucleus by promoting excessive formation of dysfunctional paraspeckles.
An H et al.
Acta Neuropathologica Communications, 2019
Xrp1 genetically interacts with the ALS-associated FUS orthologue caz and mediates its toxicity.
Mallik M et al.
The Journal of Cell Biology, 2018
Identification of rare genetic variants in Italian patients with dementia by targeted gene sequencing.
Bartoletti-Stella A et al.
Neurobiology of Aging, 2018
Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains.
Guo L et al.
Cell, 2018
Isogenic FUS-eGFP iPSC Reporter Lines Enable Quantification of FUS Stress Granule Pathology that Is Rescued by Drugs Inducing Autophagy.
Marrone L et al.
Stem Cell Reports, 2018
Mutation-dependent aggregation and toxicity in a Drosophila model for UBQLN2-associated ALS.
Kim SH et al.
Human Molecular Genetics, 2018
Neurodegeneration-associated FUS is a novel regulator of circadian gene expression.
Jiang X et al.
Translational Neurodegeneration, 2018
Mask mitigates MAPT- and FUS-induced degeneration by enhancing autophagy through lysosomal acidification.
Zhu M et al.
Autophagy, 2017
Failure to Deliver and Translate-New Insights into RNA Dysregulation in ALS.
Coyne AN et al.
Frontiers in Cellular Neuroscience, 2017
Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand.
Ramesh N et al.
Frontiers in Molecular Neuroscience, 2017
Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis.
Shang Y et al.
Brain Research, 2016
From animal models to human disease: a genetic approach for personalized medicine in ALS.
Picher-Martel V et al.
Acta Neuropathologica Communications, 2016
Pur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity.
Daigle JG et al.
Acta Neuropathologica, 2016
A systematic immunoprecipitation approach reinforces the concept of common conformational alterations in amyotrophic lateral sclerosis-linked SOD1 mutants.
Fujisawa T et al.
Neurobiology of Disease, 2015
Emerging mechanisms of molecular pathology in ALS.
Peters OM et al.
The Journal of Clinical Investigation, 2015
Mutational analysis of FUS gene and its structural and functional role in amyotrophic lateral sclerosis 6.
Kamaraj B et al.
Journal of Biomolecular Structure & Dynamics, 2015
Dissection of genetic factors associated with amyotrophic lateral sclerosis.
Leblond CS et al.
Experimental Neurology, 2014
Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms.
Shelkovnikova TA et al.
Human Molecular Genetics, 2014
The ALS gene FUS regulates synaptic transmission at the Drosophila neuromuscular junction.
Machamer JB et al.
Human Molecular Genetics, 2014
Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons.
Wang WY et al.
Nature Neuroscience, 2013