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FUS pathology in ALS is linked to alterations in multiple ALS-associated proteins and rescued by drugs stimulating autophagy

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Stress Granules in Cancer

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ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity

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Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization

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A Systematic and Comprehensive Review on Disease-Causing Genes in Amyotrophic Lateral Sclerosis

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Induced Pluripotent Stem Cells and Their Applications in Amyotrophic Lateral Sclerosis

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Dr. Jekyll and Mr. Hyde? Physiology and Pathology of Neuronal Stress Granules

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DDX17 is involved in DNA damage repair and modifies FUS toxicity in an RGG-domain dependent manner

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Defects in Axonal Transport in Inherited Neuropathies

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Viral Infections Exacerbate FUS-ALS Phenotypes in iPSC-Derived Spinal Neurons in a Virus Species-Specific Manner

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mTORC1 Crosstalk With Stress Granules in Aging and Age-Related Diseases

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Myotubularin-related phosphatase 5 is a critical determinant of autophagy in neurons

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Aberrant Stress Granule Dynamics and Aggrephagy in ALS Pathogenesis

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Studying Human Neurological Disorders Using Induced Pluripotent Stem Cells: From 2D Monolayer to 3D Organoid and Blood Brain Barrier Models

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TSC2 Interacts with HDLBP/Vigilin and Regulates Stress Granule Formation

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A Crucial Role for the Protein Quality Control System in Motor Neuron Diseases

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THRIL mediates endothelial progenitor cells autophagy via AKT pathway and FUS

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The Role of Mitophagy in Various Neurological Diseases as a Therapeutic Approach

Kaur, S., Sharma, N., Kumar, V., Sharma, D., Devi, B., Kapil, L., Singh, C., & Singh, A. (2022) · Cellular and Molecular Neurobiology · doi:10.1007/s10571-022-01302-8 · Cited by 19 · see citing works

High-throughput drug screens for amyotrophic lateral sclerosis drug discovery

McGown, A., & Stopford, M. J. (2018) · Expert Opinion on Drug Discovery · doi:10.1080/17460441.2018.1533953 · Cited by 17 · see citing works

A toolkit for the identification of NEAT1_2/paraspeckle modulators

An, H., Elvers, K. T., Gillespie, J. A., Jones, K. M., Atack, J. R., Grubisha, O., & Shelkovnikova, T. A. (2022) · Nucleic Acids Research · doi:10.1093/nar/gkac771 · Cited by 16 · see citing works

Targeting stress granules in neurodegenerative diseases: A focus on biological function and dynamics disorders

Fang, M., Liu, Y. M., Huang, C., & Fan, S. (2023) · BioFactors · doi:10.1002/biof.2017 · Cited by 16 · see citing works

Induced Pluripotent Stem Cells for Inherited Optic Neuropathies—Disease Modeling and Therapeutic Development

Harvey, J. P., Sladen, P. E., Yu‐Wai‐Man, P., & Cheetham, M. E. (2021) · Journal of Neuro-Ophthalmology · doi:10.1097/wno.0000000000001375 · Cited by 16 · see citing works

The role of autophagy in the pathogenesis and treatment of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)

Beckers, J., & Damme, P. V. (2025) · Autophagy Reports · doi:10.1080/27694127.2025.2474796 · Cited by 16 · see citing works

Induced Pluripotent Stem Cells for Inherited Optic Neuropathies—Disease Modeling and Therapeutic Development

Harvey, J., Sladen, P., Yu‐Wai‐Man, P., & Cheetham, M. E. (2022) · UCL Discovery (University College London) · Cited by 15 · see citing works

Mitochondria-lysosome-extracellular vesicles axis and nanotheranostics in neurodegenerative diseases

Kou, L., Wang, Y., Li, J., Zou, W., Jin, Z., Yin, S., Chi, X., Sun, Y., Wu, J., Wang, T., et al. (2024) · Experimental Neurology · doi:10.1016/j.expneurol.2024.114757 · Cited by 14 · see citing works

Use of CRISPR/Cas ribonucleoproteins for high throughput gene editing of induced pluripotent stem cells

Wang, Q., Chear, S., Wing, K., Stellon, D., Tran, M. T. N., Talbot, J., Pébay, A., Hewitt, A. W., & Cook, A. L. (2021) · Methods · doi:10.1016/j.ymeth.2021.02.009 · Cited by 14 · see citing works

Patient-Specific Cells for Modeling and Decoding Amyotrophic Lateral Sclerosis: Advances and Challenges

Zhao, A., Pan, Y., & Cai, S. (2020) · Stem Cell Reviews and Reports · doi:10.1007/s12015-019-09946-8 · Cited by 14 · see citing works

Pluripotent stem cells for neurodegenerative disease modeling: an expert view on their value to drug discovery

Chen, S.-D., Li, H., Cui, M., Dong, Q., & Yu, J. (2020) · Expert Opinion on Drug Discovery · doi:10.1080/17460441.2020.1767579 · Cited by 14 · see citing works

Induced Pluripotent Stem Cells in Disease Biology and the Evidence for Their In Vitro Utility

Adegunsoye, A., Gonzales, N. M., & Gilad, Y. (2023) · Annual Review of Genetics · doi:10.1146/annurev-genet-022123-090319 · Cited by 13 · see citing works

Heat resilience in embryonic zebrafish revealed using an in vivo stress granule reporter

Wang, R., Zhang, H., Du, J., & Xu, J. (2019) · Journal of Cell Science · doi:10.1242/jcs.234807 · Cited by 13 · see citing works

FUS nanoclusters are a distinct state within the dilute phase

Ge, Y., Paul, T., Gordiychuk, M., Das, N., Zhang, Y., & Myong, S. (2025) · Nature Communications · doi:10.1038/s41467-025-64909-7 · Cited by 12 · see citing works

Conformational change of RNA-helicase DHX30 by ALS/FTD-linked FUS induces mitochondrial dysfunction and cytosolic aggregates

Hikiami, R., Morimura, T., Ayaki, T., Tsukiyama, T., Morimura, N., Kusui, M., Wada, H., Minamiyama, S., Shodai, A., Asada‐Utsugi, M., et al. (2022) · Scientific Reports · doi:10.1038/s41598-022-20405-2 · Cited by 12 · see citing works

Rapid and Robust Multi-Phenotypic Assay System for ALS Using Human iPS Cells with Mutations in Causative Genes

Kondo, T., Ebinuma, I., Tanaka, H., Nishikawa, Y., Komiya, T., Ishikawa, M., & Okano, H. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms24086987 · Cited by 12 · see citing works

BAG3 and BAG6 differentially affect the dynamics of stress granules by targeting distinct subsets of defective polypeptides released from ribosomes

Mediani, L., Galli, V., Carrà, A. D., Bigi, I., Vinet, J., Ganassi, M., Antoniani, F., Tiago, T., Cimino, M., Matějů, D., et al. (2020) · Cell Stress and Chaperones · doi:10.1007/s12192-020-01141-w · Cited by 12 · see citing works

Motor neuron-derived induced pluripotent stem cells as a drug screening platform for amyotrophic lateral sclerosis

Amorós, M. A., Choi, E. S., Cofré, A. H. R., Dokholyan, N. V., & Duzzioni, M. (2022) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2022.962881 · Cited by 11 · see citing works

Identifying FUS amyotrophic lateral sclerosis disease signatures in patient dermal fibroblasts

Kumbier, K., Roth, M. A., Li, Z., Lazzari-Dean, J. R., Waters, C., Hammerlindl, S., Rinaldi, C., Huang, P., Korben, V. A., Phatnani, H. P., et al. (2024) · Developmental Cell · doi:10.1016/j.devcel.2024.05.011 · Cited by 10 · see citing works

Live Cell Imaging of ATP Levels Reveals Metabolic Compartmentalization within Motoneurons and Early Metabolic Changes in FUS ALS Motoneurons

Zimyanin, V. L., Pielka, A.-M., Glaß, H., Japtok, J., Großmann, D., Martin, M., Deußen, A., Szewczyk, B., Deppmann, C. D., Zunder, E. R., et al. (2023) · Cells · doi:10.3390/cells12101352 · Cited by 9 · see citing works

Digital color-coded molecular barcoding reveals dysregulation of common FUS and FMRP targets in soma and neurites of ALS mutant motoneurons

Garone, M. G., Salerno, D., & Rosa, A. (2023) · Cell Death Discovery · doi:10.1038/s41420-023-01340-1 · Cited by 8 · see citing works

Selective Reduction of Ca2+ Entry Through the Human NMDA Receptor: a Quantitative Study by Simultaneous Ca2+ and Na+ Imaging

D’Andrea, T., Benedetti, M. C., Monaco, L., Rosa, A., & Fucile, S. (2024) · Molecular Neurobiology · doi:10.1007/s12035-024-03944-9 · Cited by 6 · see citing works

Autophagy in motor neuron diseases

Rodríguez‐Muela, N. (2020) · Progress in molecular biology and translational science · doi:10.1016/bs.pmbts.2020.03.009 · Cited by 6 · see citing works

Functional genomics, genetic risk profiling and cell phenotypes in neurodegenerative disease

Finkbeiner, S. M. (2020) · Neurobiology of Disease · doi:10.1016/j.nbd.2020.105088 · Cited by 6 · see citing works

The Role of iPSC Modeling Toward Projection of Autophagy Pathway in Disease Pathogenesis: Leader or Follower

Kolahdouzmohammadi, M., Totonchi, M., & Pahlavan, S. (2020) · Stem Cell Reviews and Reports · doi:10.1007/s12015-020-10077-8 · Cited by 6 · see citing works

Disruption of RNA-binding proteins in neurological disorders

Huang, L., Zhao, B., & Wan, Y. (2024) · Experimental Neurology · doi:10.1016/j.expneurol.2024.115119 · Cited by 5 · see citing works

Propranolol reduces the accumulation of cytotoxic aggregates in C9orf72-ALS/FTD in vitro models

Seidel, M., Rajkumar, S., Steffke, C., Noeth, V., Agarwal, S., Roger, K., Lipecka, J., Ludolph, A. C., Guerrera, I. C., Boeckers, T. M., et al. (2023) · Current Research in Neurobiology · doi:10.1016/j.crneur.2023.100105 · Cited by 5 · see citing works

Obatoclax Rescues FUS-ALS Phenotypes in iPSC-Derived Neurons by Inducing Autophagy

Bautista, C. M. C., Eismann, K., Gentzel, M., Pelucchi, S., Mertens, J., Walters, H., Yun, M. H., & Sterneckert, J. L. (2023) · Cells · doi:10.3390/cells12182247 · Cited by 4 · see citing works

Protein Aggregation in Amyotrophic Lateral Sclerosis

Chisholm, C. G., Yerbury, J. J., & McAlary, L. (2021) · Wiley eBooks · doi:10.1002/9781119745532.ch6 · Cited by 5 · see citing works

Hyperosmotic Stress Induces a Specific Pattern for Stress Granule Formation in Human-Induced Pluripotent Stem Cells

Salloum‐Asfar, S., Engelke, R., Mousa, H., Goswami, N., Thompson, I. R., Palangi, F., Kamal, K., Al-Noubi, M. N., Schmidt, F., Abdulla, S. A., et al. (2021) · Stem Cells International · doi:10.1155/2021/8274936 · Cited by 3 · see citing works

A Human iPSC Line Carrying a de novo Pathogenic FUS Mutation Identified in a Patient With Juvenile ALS Differentiated Into Motor Neurons With Pathological Characteristics

Chen, L., Wang, Y., & Xie, J. (2020) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2020.00273 · Cited by 3 · see citing works

Human J-domain proteins promote stress granule disassembly and suppress neurodegeneration-linked protein aggregation

Mastromarco, G. J., Earnshaw, R., Moore, G., Xu, X. Y. S., Sadek, N. H., Cui, F., Lo, N., & Lee, H. O. (2026) · Cell Reports · doi:10.1016/j.celrep.2026.117326 · Cited by 2 · see citing works

Proteomic signature and mesenchymal stromal/stem cell-derived extracellular vesicle treatment of iPSC motor neurons from different ALS patient groupsPreprint

Varderidou‐Minasian, S., Jakobs, C. E., Pasteuning-Vuhman, S., Timmers, A., Altelaar, M. A. F., Lorenowicz, M. J., & Pasterkamp, R. J. (2022) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2022.07.05.498816 · Cited by 2 · see citing works

Axonal transcriptome reveals upregulation of PLK1 as a protective mechanism in response to increased DNA damage in FUS P525L spinal motor neuronsPreprint

Zimyanin, V. L., Dash, B. P., Großmann, D., Simolka, T., Glaß, H., Verma, R., Khatri, V., Deppmann, C. D., Zunder, E. R., Redemann, S., et al. (2024) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2024.11.20.624439 · Cited by 2 · see citing works

Reporter Alleles in hiPSCs: Visual Cues on Development and Disease

Cotta, G. C., dos Santos, R. C. T., Costa, G. M. J., & Lacerda, S. M. D. S. N. (2024) · International Journal of Molecular Sciences · doi:10.3390/ijms252011009 · Cited by 1 · see citing works

Compartment-specific transcriptome of motor neurons reveals impaired extracellular matrix signaling and activated cell cycle kinases in FUS-ALS

Zimyanin, V. L., Dash, B. P., Simolka, T., Glaß, H., Pal, A., Haidle, F., Zarnack, K., Verma, R., Khatri, V., Deppmann, C. D., et al. (2026) · Neurobiology of Disease · doi:10.1016/j.nbd.2026.107268 · Cited by 1 · see citing works

Granules Gone Rogue: Nuclear and Cytoplasmic Ribonucleoprotein Structures in Amyotrophic Lateral Sclerosis-Fused in Sarcoma (ALS-FUS) Pathology

Ahuja, V., Sahu, B., Khurana, S., Kumari, K., Ganguly, N. K., Gourie‐Devi, M., Verma, S., Dastidar, S. G., & Taneja, V. (2026) · Molecular Neurobiology · doi:10.1007/s12035-026-05736-9 · Cited by 1 · see citing works

ALS-FUS mutation affects the activities of HuD/ELAVL4 and FMRP leading to axon phenotypes in motoneuronsPreprint

Garone, M. G., Birsa, N., Rosito, M., Salaris, F., Mochi, M., de Turris, V., Nair, R., Cunningham, T. J., Fisher, E., Morlando, M., et al. (2020) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2020.08.26.268631 · Cited by 1 · see citing works

A scalable screening platform for phenotypic subtyping of ALS patient-derived fibroblastsPreprint

Kumbier, K., Roth, M. A., Li, Z., Lazzari-Dean, J. R., Waters, C., Huang, P., Korobeynikov, V. A., Phatnani, H. P., Shneider, N. A., Jacobson, M. P., et al. (2022) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2022.09.27.509770 · Cited by 1 · see citing works

Unravelling novel functions of the MTOR network across subcellular compartments

Sandoval, M. C. (2022) · doi:10.33612/diss.208570309

Platform Communications: Abstract Book - 31st International Symposium on ALS/MND (Complete printable file)

Crosio, C. (2020) · Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration · doi:10.1080/21678421.2020.1828775

A função de proteínas associadas à Esclerose Lateral Amiotrófica em diferentes contextos celulares

Assoni, A. F. (2022) · doi:10.11606/t.41.2022.tde-02082022-190632

Gene editing hPSCs for modeling neurological disorders

Petersen, A. J. (2022) · Elsevier eBooks · doi:10.1016/b978-0-12-822277-5.00014-6

Autophagy regulates amyotrophic lateral sclerosis-linked fused in sarcoma-positive stress granules in neurons

Ryu, H.-H., Jun, M.-H., Min, K.-J., Jang, D.-J., Lee, Y.-S., Kim, H. K., & Lee, J.-A. (2014) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2014.07.026 · Cited by 125 · see citing works

ALS mutant FUS proteins are recruited into stress granules in induced Pluripotent Stem Cells (iPSCs) derived motoneurons

Lenzi, J., Santis, R. D., de Turris, V., Morlando, M., Laneve, P., Calvo, A., Caliendo, V., Chiò, A., Rosa, A., & Bozzoni, I. (2015) · Disease Models & Mechanisms · doi:10.1242/dmm.020099 · Cited by 140 · see citing works

Autophagy meets fused in sarcoma-positive stress granules

Matus, S., Bosco, D. A., & Hetz, C. A. (2014) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2014.08.019 · Cited by 15 · see citing works

The fused in sarcoma protein forms cytoplasmic aggregates in motor neurons derived from integration-free induced pluripotent stem cells generated from a patient with familial amyotrophic lateral sclerosis carrying the FUS-P525L mutation

Liu, X., Chen, J., Liu, W., Li, X., Chen, Q., Liu, T., Gao, S., & Deng, M. (2015) · Neurogenetics · doi:10.1007/s10048-015-0448-y · Cited by 32 · see citing works

Disrupted autophagy and neuronal dysfunction in C. elegans knockin models of FUS amyotrophic lateral sclerosis

Baskoylu, S., Chapkis, N., Unsal, B., Lins, J., Schuch, K. N., Simon, J., & Hart, A. C. (2022) · Cell Reports · doi:10.1016/j.celrep.2021.110195 · Cited by 37 · see citing works