Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation
Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disease. Cytoplasmic fused in sarcoma (FUS) aggregates are pathological hallmarks of FUS-ALS. Proper shuttling between the nucleus and cytoplasm is essential for physiological cell function. However, the initial event in the patho...
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| Published in | Nature communications Vol. 9; no. 1; pp. 335 - 17 |
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| Main Authors | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
London
Nature Publishing Group UK
23.01.2018
Nature Publishing Group Nature Portfolio |
| Subjects | |
| Online Access | Get full text |
| ISSN | 2041-1723 2041-1723 |
| DOI | 10.1038/s41467-017-02299-1 |
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| Abstract | Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disease. Cytoplasmic fused in sarcoma (FUS) aggregates are pathological hallmarks of FUS-ALS. Proper shuttling between the nucleus and cytoplasm is essential for physiological cell function. However, the initial event in the pathophysiology of FUS-ALS remains enigmatic. Using human induced pluripotent stem cell (hiPSCs)-derived motor neurons (MNs), we show that impairment of poly(ADP-ribose) polymerase (PARP)-dependent DNA damage response (DDR) signaling due to mutations in the FUS nuclear localization sequence (NLS) induces additional cytoplasmic FUS mislocalization which in turn results in neurodegeneration and FUS aggregate formation. Our work suggests that a key pathophysiologic event in ALS is upstream of aggregate formation. Targeting DDR signaling could lead to novel therapeutic routes for ameliorating ALS.
Abnormal cytoplasmic aggregates of FUS are a hallmark of some forms of amyotrophic lateral sclerosis (ALS). Here, using neurons derived from patients with FUS-ALS, the authors demonstrate that impairment of PARP-dependent DNA damage signaling is an event that occurs upstream of neurodegeneration and cytoplasmic aggregate formation in FUS-ALS. |
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| AbstractList | Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disease. Cytoplasmic fused in sarcoma (FUS) aggregates are pathological hallmarks of FUS-ALS. Proper shuttling between the nucleus and cytoplasm is essential for physiological cell function. However, the initial event in the pathophysiology of FUS-ALS remains enigmatic. Using human induced pluripotent stem cell (hiPSCs)-derived motor neurons (MNs), we show that impairment of poly(ADP-ribose) polymerase (PARP)-dependent DNA damage response (DDR) signaling due to mutations in the FUS nuclear localization sequence (NLS) induces additional cytoplasmic FUS mislocalization which in turn results in neurodegeneration and FUS aggregate formation. Our work suggests that a key pathophysiologic event in ALS is upstream of aggregate formation. Targeting DDR signaling could lead to novel therapeutic routes for ameliorating ALS. Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disease. Cytoplasmic fused in sarcoma (FUS) aggregates are pathological hallmarks of FUS-ALS. Proper shuttling between the nucleus and cytoplasm is essential for physiological cell function. However, the initial event in the pathophysiology of FUS-ALS remains enigmatic. Using human induced pluripotent stem cell (hiPSCs)-derived motor neurons (MNs), we show that impairment of poly(ADP-ribose) polymerase (PARP)-dependent DNA damage response (DDR) signaling due to mutations in the FUS nuclear localization sequence (NLS) induces additional cytoplasmic FUS mislocalization which in turn results in neurodegeneration and FUS aggregate formation. Our work suggests that a key pathophysiologic event in ALS is upstream of aggregate formation. Targeting DDR signaling could lead to novel therapeutic routes for ameliorating ALS.Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disease. Cytoplasmic fused in sarcoma (FUS) aggregates are pathological hallmarks of FUS-ALS. Proper shuttling between the nucleus and cytoplasm is essential for physiological cell function. However, the initial event in the pathophysiology of FUS-ALS remains enigmatic. Using human induced pluripotent stem cell (hiPSCs)-derived motor neurons (MNs), we show that impairment of poly(ADP-ribose) polymerase (PARP)-dependent DNA damage response (DDR) signaling due to mutations in the FUS nuclear localization sequence (NLS) induces additional cytoplasmic FUS mislocalization which in turn results in neurodegeneration and FUS aggregate formation. Our work suggests that a key pathophysiologic event in ALS is upstream of aggregate formation. Targeting DDR signaling could lead to novel therapeutic routes for ameliorating ALS. Abnormal cytoplasmic aggregates of FUS are a hallmark of some forms of amyotrophic lateral sclerosis (ALS). Here, using neurons derived from patients with FUS-ALS, the authors demonstrate that impairment of PARP-dependent DNA damage signaling is an event that occurs upstream of neurodegeneration and cytoplasmic aggregate formation in FUS-ALS. Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disease. Cytoplasmic fused in sarcoma (FUS) aggregates are pathological hallmarks of FUS-ALS. Proper shuttling between the nucleus and cytoplasm is essential for physiological cell function. However, the initial event in the pathophysiology of FUS-ALS remains enigmatic. Using human induced pluripotent stem cell (hiPSCs)-derived motor neurons (MNs), we show that impairment of poly(ADP-ribose) polymerase (PARP)-dependent DNA damage response (DDR) signaling due to mutations in the FUS nuclear localization sequence (NLS) induces additional cytoplasmic FUS mislocalization which in turn results in neurodegeneration and FUS aggregate formation. Our work suggests that a key pathophysiologic event in ALS is upstream of aggregate formation. Targeting DDR signaling could lead to novel therapeutic routes for ameliorating ALS. Abnormal cytoplasmic aggregates of FUS are a hallmark of some forms of amyotrophic lateral sclerosis (ALS). Here, using neurons derived from patients with FUS-ALS, the authors demonstrate that impairment of PARP-dependent DNA damage signaling is an event that occurs upstream of neurodegeneration and cytoplasmic aggregate formation in FUS-ALS. |
| ArticleNumber | 335 |
| Author | Storch, Alexander Ludolph, Albert C. Petri, Susanne Holzmann, Karlheinz Vehlow, Anne Lojewski, Xenia Frickenhaus, Marie Storkebaum, Erik Pal, Arun Weis, Joachim Boeckers, Tobias M. Stanslowsky, Nancy Hyman, Anthony A. Freischmidt, Axel Cordes, Nils Sterneckert, Jared Naumann, Marcel Poser, Ina Troost, Dirk Grill, Stephan W. Japtok, Julia Weishaupt, Jochen H. Hermann, Andreas Pan-Montojo, Francisco Naujock, Maximilian Wegner, Florian Goswami, Anand Liebau, Stefan Günther, René Jin, Mengmeng Reinhardt, Peter |
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KG – sequence: 8 givenname: René surname: Günther fullname: Günther, René organization: Department of Neurology, Technische Universität Dresden – sequence: 9 givenname: Mengmeng surname: Jin fullname: Jin, Mengmeng organization: Department of Neurology, Technische Universität Dresden – sequence: 10 givenname: Nancy surname: Stanslowsky fullname: Stanslowsky, Nancy organization: Department of Neurology, Hannover Medical School – sequence: 11 givenname: Peter surname: Reinhardt fullname: Reinhardt, Peter organization: Center for Regenerative Therapies Dresden (CRTD), Technische Universität Dresden, AbbVie Deutschland GmbH & Co. KG – sequence: 12 givenname: Jared surname: Sterneckert fullname: Sterneckert, Jared organization: Center for Regenerative Therapies Dresden (CRTD), Technische Universität Dresden – sequence: 13 givenname: Marie surname: Frickenhaus fullname: Frickenhaus, Marie organization: Molecular Neurogenetics Laboratory, Max Planck Institute for Molecular Biomedicine, Faculty of Medicine, University of Münster – sequence: 14 givenname: Francisco surname: Pan-Montojo fullname: Pan-Montojo, Francisco organization: Department of Neurology, Klinikum der Universität München, and Munich Cluster for Systems Neurology – sequence: 15 givenname: Erik surname: Storkebaum fullname: Storkebaum, Erik organization: Molecular Neurogenetics Laboratory, Max Planck Institute for Molecular Biomedicine, Faculty of Medicine, University of Münster, Department of Molecular Neurobiology, Donders Institute for Brain, Cognition and Behaviour, Radboud University – sequence: 16 givenname: Ina surname: Poser fullname: Poser, Ina organization: Max Planck Institute of Molecular Cell Biology and Genetics – sequence: 17 givenname: Axel surname: Freischmidt fullname: Freischmidt, Axel organization: Department of Neurology, University Ulm – sequence: 18 givenname: Jochen H. surname: Weishaupt fullname: Weishaupt, Jochen H. organization: Department of Neurology, University Ulm – sequence: 19 givenname: Karlheinz surname: Holzmann fullname: Holzmann, Karlheinz organization: Genomics-Core Facility, University Hospital Ulm, Centre for Biomedical Research – sequence: 20 givenname: Dirk surname: Troost fullname: Troost, Dirk organization: Division of Neuropathology, Department of Pathology, Academic Medical Centre – sequence: 21 givenname: Albert C. surname: Ludolph fullname: Ludolph, Albert C. organization: Department of Neurology, University Ulm – sequence: 22 givenname: Tobias M. surname: Boeckers fullname: Boeckers, Tobias M. organization: Institute of Anatomy and Cell Biology, University of Ulm – sequence: 23 givenname: Stefan surname: Liebau fullname: Liebau, Stefan organization: Institute of Neuroanatomy & Developmental Biology, Eberhard Karls University of Tübingen – sequence: 24 givenname: Susanne surname: Petri fullname: Petri, Susanne organization: Department of Neurology, Hannover Medical School – sequence: 25 givenname: Nils surname: Cordes fullname: Cordes, Nils organization: OncoRay—National Center for Radiation Research in Oncology, Faculty of Medicine and University Hospital Carl Gustav Carus, Technische Universität Dresden, Helmholtz-Zentrum Dresden-Rossendorf, German Cancer Consortium (DKTK), partner site Dresden, and German Cancer Research Center (DKFZ), Helmholtz-Zentrum Dresden-Rossendorf, Institute of Radiooncology—OncoRay, Department of Radiotherapy and Radiation Oncology, Faculty of Medicine and University Hospital Carl Gustav Carus, Technische Universität Dresden – sequence: 26 givenname: Anthony A. surname: Hyman fullname: Hyman, Anthony A. organization: Max Planck Institute of Molecular Cell Biology and Genetics – sequence: 27 givenname: Florian surname: Wegner fullname: Wegner, Florian organization: Department of Neurology, Hannover Medical School – sequence: 28 givenname: Stephan W. surname: Grill fullname: Grill, Stephan W. organization: Max Planck Institute of Molecular Cell Biology and Genetics, BIOTEC, Technische Universität Dresden – sequence: 29 givenname: Joachim surname: Weis fullname: Weis, Joachim organization: Institute of Neuropathology, RWTH Aachen University Hospital – sequence: 30 givenname: Alexander surname: Storch fullname: Storch, Alexander organization: Department of Neurology, Technische Universität Dresden, Center for Regenerative Therapies Dresden (CRTD), Technische Universität Dresden, German Center for Neurodegenerative Diseases (DZNE), Research Site Rostock, Department of Neurology, University of Rostock – sequence: 31 givenname: Andreas surname: Hermann fullname: Hermann, Andreas email: Andreas.Hermann@uniklinikum-dresden.de organization: Department of Neurology, Technische Universität Dresden, Center for Regenerative Therapies Dresden (CRTD), Technische Universität Dresden, German Center for Neurodegenerative Diseases (DZNE), Research Site Rostock |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/29362359$$D View this record in MEDLINE/PubMed |
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| ContentType | Journal Article |
| Copyright | The Author(s) 2018 2018. This work is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License. |
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| DOI | 10.1038/s41467-017-02299-1 |
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| Snippet | Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disease. Cytoplasmic fused in sarcoma (FUS) aggregates are pathological hallmarks of... Abnormal cytoplasmic aggregates of FUS are a hallmark of some forms of amyotrophic lateral sclerosis (ALS). Here, using neurons derived from patients with... |
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| SubjectTerms | 631/337/1427/2566 631/378/1689/1285 Adenosine diphosphate Amyotrophic lateral sclerosis Cytoplasm Damage localization Deoxyribonucleic acid DNA DNA damage FUS protein Humanities and Social Sciences Localization Motor neuron diseases multidisciplinary Mutation Neurodegeneration Nuclei (cytology) Pathophysiology Pluripotency Poly(ADP-ribose) Poly(ADP-ribose) polymerase Ribose Sarcoma Science Science (multidisciplinary) Signaling Stem cells |
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| Title | Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation |
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