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Wyszukujesz frazę ""RNA-Binding Protein FUS"" wg kryterium: Temat


Tytuł:
Zebrafish CCNF and FUS Mediate Stress-Specific Motor Responses.
Autorzy:
Aksoy YA; Sydney Medical School, Faculty of Medicine and Health, The University of Sydney, Sydney, NSW 2006, Australia.; Cancer Diagnosis and Pathology Group, Kolling Institute of Medical Research, Royal North Shore Hospital, St Leonards, NSW 2065, Australia.; Macquarie Medical School, Macquarie University, Sydney, NSW 2109, Australia.
Cole AJ; Centenary Institute, Faculty of Medicine and Health, The University of Sydney, Sydney, NSW 2006, Australia.
Deng W; School of Biomedical Engineering, University of Technology Sydney, Ultimo, NSW 2007, Australia.
Hesselson D; Centenary Institute, Faculty of Medicine and Health, The University of Sydney, Sydney, NSW 2006, Australia.
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Źródło:
Cells [Cells] 2024 Feb 21; Vol. 13 (5). Date of Electronic Publication: 2024 Feb 21.
Typ publikacji:
Journal Article
MeSH Terms:
Amyotrophic Lateral Sclerosis*/metabolism
Cyclins*/metabolism
Neurodegenerative Diseases*/metabolism
RNA-Binding Protein FUS*/genetics
RNA-Binding Protein FUS*/metabolism
Zebrafish*/metabolism
Animals ; Humans ; Motor Neurons/pathology ; Proteins/metabolism
Czasopismo naukowe
Tytuł:
Neuronal dysfunction caused by FUSR521G promotes ALS-associated phenotypes that are attenuated by NF-κB inhibition.
Autorzy:
Pelaez MC; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Desmeules A; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Gelon PA; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Glasson B; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Marcadet L; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Rodgers A; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Phaneuf D; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Pozzi S; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Dutchak PA; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Julien JP; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada.
Sephton CF; Department of Psychiatry and Neuroscience, CERVO Brain Research Centre, Laval University, Quebec City, QC, Canada. .
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Źródło:
Acta neuropathologica communications [Acta Neuropathol Commun] 2023 Nov 16; Vol. 11 (1), pp. 182. Date of Electronic Publication: 2023 Nov 16.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*/pathology
Frontotemporal Dementia*/pathology
Aged ; Animals ; Humans ; Mice ; Disease Progression ; Mice, Transgenic ; Motor Neurons/metabolism ; Mutation ; NF-kappa B/metabolism ; RNA-Binding Protein FUS/genetics ; RNA-Binding Protein FUS/metabolism
Czasopismo naukowe
Tytuł:
lncRNA Sequencing Reveals Neurodegeneration-Associated FUS Mutations Alter Transcriptional Landscape of iPS Cells That Persists in Motor Neurons.
Autorzy:
Provasek VE; Division of DNA Repair Research within the Center for Neuroregeneration, Department of Neurosurgery, Houston Methodist Research Institute, Houston, TX 77030, USA.; School of Medicine, Texas A&M University, College Station, TX 77843, USA.
Kodavati M; Division of DNA Repair Research within the Center for Neuroregeneration, Department of Neurosurgery, Houston Methodist Research Institute, Houston, TX 77030, USA.
Guo W; INSERM, UMR-S1118, Mécanismes Centraux et Périphériques de la Neurodégénérescence, Université de Strasbourg, CRBS, 67000 Strasbourg, France.; VIB, Center for Brain & Disease Research, 3000 Leuven, Belgium.; Leuven Brain Institute (LBI), 3000 Leuven, Belgium.; Stem Cell Institute, Department of Development and Regeneration, KU Leuven, 3000 Leuven, Belgium.
Wang H; Division of DNA Repair Research within the Center for Neuroregeneration, Department of Neurosurgery, Houston Methodist Research Institute, Houston, TX 77030, USA.
Boldogh I; Department of Microbiology and Immunology, University of Texas Medical Branch, Galveston, TX 77555, USA.
Van Den Bosch L; Stem Cell Institute, Department of Development and Regeneration, KU Leuven, 3000 Leuven, Belgium.
Britz G; Department of Neurosurgery, Houston Methodist Research Institute, Houston, TX 77030, USA.
Hegde ML; Division of DNA Repair Research within the Center for Neuroregeneration, Department of Neurosurgery, Houston Methodist Research Institute, Houston, TX 77030, USA.; School of Medicine, Texas A&M University, College Station, TX 77843, USA.; Department of Neurosurgery, Weill Cornell Medical College, New York, NY 10065, USA.
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Źródło:
Cells [Cells] 2023 Oct 16; Vol. 12 (20). Date of Electronic Publication: 2023 Oct 16.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't; Research Support, N.I.H., Extramural
MeSH Terms:
Amyotrophic Lateral Sclerosis*/genetics
Amyotrophic Lateral Sclerosis*/metabolism
Induced Pluripotent Stem Cells*/metabolism
RNA, Long Noncoding*/genetics
RNA, Long Noncoding*/metabolism
Humans ; Motor Neurons/metabolism ; Mutation/genetics ; RNA-Binding Protein FUS/genetics ; RNA-Binding Protein FUS/metabolism
Czasopismo naukowe
Tytuł:
Obatoclax Rescues FUS-ALS Phenotypes in iPSC-Derived Neurons by Inducing Autophagy.
Autorzy:
Castillo Bautista CM; Center for Regenerative Therapies TU Dresden (CRTD), Technische Universität Dresden, 01307 Dresden, Germany.
Eismann K; Core Facility Mass Spectrometry & Proteomics, Center for Molecular and Cellular Bioengineering, Technische Universität Dresden, 01307 Dresden, Germany.
Gentzel M; Core Facility Mass Spectrometry & Proteomics, Center for Molecular and Cellular Bioengineering, Technische Universität Dresden, 01307 Dresden, Germany.
Pelucchi S; Department of Neurosciences, University of California San Diego, La Jolla, CA 92161, USA.; Department of Pharmacological and Biomolecular Sciences, Università degli Studi di Milano, 20133 Milan, Italy.
Mertens J; Department of Neurosciences, University of California San Diego, La Jolla, CA 92161, USA.; Laboratory of Genetics, The Salk Institute for Biological Studies, La Jolla, CA 92037, USA.; Institute for Molecular Biology, University of Innsbruck, A-6020 Innsbruck, Austria.
Walters HE; Center for Regenerative Therapies TU Dresden (CRTD), Technische Universität Dresden, 01307 Dresden, Germany.
Yun MH; Center for Regenerative Therapies TU Dresden (CRTD), Technische Universität Dresden, 01307 Dresden, Germany.; Max Planck Institute for Molecular Cell Biology and Genetics, 01307 Dresden, Germany.; Cluster of Excellence Physics of Life, Technische Universität Dresden, 01307 Dresden, Germany.
Sterneckert J; Center for Regenerative Therapies TU Dresden (CRTD), Technische Universität Dresden, 01307 Dresden, Germany.; Medical Faculty Carl Gustav Carus of TU Dresden, 01307 Dresden, Germany.
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Źródło:
Cells [Cells] 2023 Sep 11; Vol. 12 (18). Date of Electronic Publication: 2023 Sep 11.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*/metabolism
Induced Pluripotent Stem Cells*/metabolism
Humans ; Motor Neurons/metabolism ; Mutation ; Autophagy/physiology ; Phenotype ; Proto-Oncogene Proteins c-bcl-2/metabolism ; RNA-Binding Protein FUS/genetics ; RNA-Binding Protein FUS/metabolism
Czasopismo naukowe
Tytuł:
Benchmarking of force fields to characterize the intrinsically disordered R2-FUS-LC region.
Autorzy:
Chan-Yao-Chong M; Molecular Modeling and Simulation (MMS) Team, Institute for Quantum Life Science, National Institutes for Quantum Science and Technology (QST), 4-9-1, Anagawa, Inage Ward, Chiba City, Chiba, 263-8555, Japan.; Toulouse Biotechnology Institute, TBI, Université de Toulouse, CNRS, INRAE, INSA, 135, Avenue de Rangueil, 31077, Toulouse Cedex 04, France.
Chan J; Molecular Modeling and Simulation (MMS) Team, Institute for Quantum Life Science, National Institutes for Quantum Science and Technology (QST), 4-9-1, Anagawa, Inage Ward, Chiba City, Chiba, 263-8555, Japan.
Kono H; Molecular Modeling and Simulation (MMS) Team, Institute for Quantum Life Science, National Institutes for Quantum Science and Technology (QST), 4-9-1, Anagawa, Inage Ward, Chiba City, Chiba, 263-8555, Japan. .
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Źródło:
Scientific reports [Sci Rep] 2023 Aug 30; Vol. 13 (1), pp. 14226. Date of Electronic Publication: 2023 Aug 30.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*
Intrinsically Disordered Proteins*
Humans ; Benchmarking ; Water ; RNA-Binding Protein FUS
Czasopismo naukowe
Tytuł:
Restoring Axonal Organelle Motility and Regeneration in Cultured FUS-ALS Motoneurons through Magnetic Field Stimulation Suggests an Alternative Therapeutic Approach.
Autorzy:
Kandhavivorn W; Dresden High Magnetic Field Laboratory (HLD-EMFL), Helmholtz-Zentrum Dresden-Rossendorf, D-01328 Dresden, Germany.; Institute of Anatomy, Technische Universität Dresden, D-01307 Dresden, Germany.
Glaß H; Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, D-01307 Dresden, Germany.; Translational Neurodegeneration Section 'Albrecht Kossel', Department of Neurology, University Medical Center Rostock, University of Rostock, D-18147 Rostock, Germany.
Herrmannsdörfer T; Dresden High Magnetic Field Laboratory (HLD-EMFL), Helmholtz-Zentrum Dresden-Rossendorf, D-01328 Dresden, Germany.
Böckers TM; Institute of Anatomy and Cell Biology, University of Ulm, D-89081 Ulm, Germany.; Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE) Ulm, D-89081 Ulm, Germany.
Uhlarz M; Dresden High Magnetic Field Laboratory (HLD-EMFL), Helmholtz-Zentrum Dresden-Rossendorf, D-01328 Dresden, Germany.
Gronemann J; Dresden High Magnetic Field Laboratory (HLD-EMFL), Helmholtz-Zentrum Dresden-Rossendorf, D-01328 Dresden, Germany.
Funk RHW; Institute of Anatomy, Technische Universität Dresden, D-01307 Dresden, Germany.; Dresden International University, D-01067 Dresden, Germany.
Pietzsch J; Department of Radiopharmaceutical and Chemical Biology, Institute of Radiopharmaceutical Cancer Research, Helmholtz-Zentrum Dresden-Rossendorf, D-01328 Dresden, Germany.; Faculty of Chemistry and Food Chemistry, School of Science, Technische Universität Dresden, D-01069 Dresden, Germany.
Pal A; Dresden High Magnetic Field Laboratory (HLD-EMFL), Helmholtz-Zentrum Dresden-Rossendorf, D-01328 Dresden, Germany.; Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, D-01307 Dresden, Germany.
Hermann A; Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, D-01307 Dresden, Germany.; Translational Neurodegeneration Section 'Albrecht Kossel', Department of Neurology, University Medical Center Rostock, University of Rostock, D-18147 Rostock, Germany.; Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE) Rostock/Greifswald, D-18147 Rostock, Germany.; Center for Transdisciplinary Neurosciences Rostock (CTNR), University Medical Center Rostock, University of Rostock, D-18147 Rostock, Germany.
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Źródło:
Cells [Cells] 2023 May 29; Vol. 12 (11). Date of Electronic Publication: 2023 May 29.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*/metabolism
Humans ; Motor Neurons/pathology ; Axons/metabolism ; Organelles/metabolism ; Magnetic Fields ; RNA-Binding Protein FUS/genetics ; RNA-Binding Protein FUS/metabolism
Czasopismo naukowe
Tytuł:
Live Cell Imaging of ATP Levels Reveals Metabolic Compartmentalization within Motoneurons and Early Metabolic Changes in FUS ALS Motoneurons.
Autorzy:
Zimyanin VL; Department of Molecular Physiology and Biological Physics, School of Medicine, University of Virginia, Charlottesville, VA 22903, USA.; Center for Membrane and Cell Physiology, School of Medicine, University of Virginia, Charlottesville, VA 22903, USA.; Department of Neurology, Technische Universität Dresden, 01307 Dresden, Germany.
Pielka AM; Translational Neurodegeneration Section, 'Albrecht Kossel', Department of Neurology, University Medical Center Rostock, University of Rostock, 18147 Rostock, Germany.
Glaß H; Translational Neurodegeneration Section, 'Albrecht Kossel', Department of Neurology, University Medical Center Rostock, University of Rostock, 18147 Rostock, Germany.
Japtok J; Department of Neurology, Technische Universität Dresden, 01307 Dresden, Germany.
Großmann D; Translational Neurodegeneration Section, 'Albrecht Kossel', Department of Neurology, University Medical Center Rostock, University of Rostock, 18147 Rostock, Germany.
Martin M; Institute of Physiology, Technische Universität Dresden, 01307 Dresden, Germany.
Deussen A; Institute of Physiology, Technische Universität Dresden, 01307 Dresden, Germany.
Szewczyk B; Translational Neurodegeneration Section, 'Albrecht Kossel', Department of Neurology, University Medical Center Rostock, University of Rostock, 18147 Rostock, Germany.
Deppmann C; Department of Biology, Graduate School of Arts and Sciences, University of Virginia, Charlottesville, VA 22902, USA.
Zunder E; Department of Biomedical Engineering, School of Medicine, University of Virginia, Charlottesville, VA 22902, USA.
Andersen PM; Department of Clinical Sciences, Neurosciences, Umeå University, SE-901 85 Umeå, Sweden.
Boeckers TM; Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE), Ulm Site, 89081 Ulm, Germany.; Institute for Anatomy and Cell Biology, Ulm University, 89081 Ulm, Germany.
Sterneckert J; Centre for Regenerative Therapie, Technische Universität Dresden, 01307 Dresden, Germany.; Medical Faculty Carl Gustav Carus, Technische Universität Dresden, 01307 Dresden, Germany.
Redemann S; Department of Molecular Physiology and Biological Physics, School of Medicine, University of Virginia, Charlottesville, VA 22903, USA.; Center for Membrane and Cell Physiology, School of Medicine, University of Virginia, Charlottesville, VA 22903, USA.; Department of Cell Biology, School of Medicine, University of Virginia, Charlottesville, VA 22902, USA.
Storch A; Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE) Rostock/Greifswald, 18147 Rostock, Germany.; Center for Transdisciplinary Neurosciences Rostock (CTNR), University Medical Centre, University of Rostock, 18147 Rostock, Germany.; Department of Neurology, University of Rostock, 18147 Rostock, Germany.
Hermann A; Translational Neurodegeneration Section, 'Albrecht Kossel', Department of Neurology, University Medical Center Rostock, University of Rostock, 18147 Rostock, Germany.; Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE) Rostock/Greifswald, 18147 Rostock, Germany.; Center for Transdisciplinary Neurosciences Rostock (CTNR), University Medical Centre, University of Rostock, 18147 Rostock, Germany.
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Źródło:
Cells [Cells] 2023 May 09; Vol. 12 (10). Date of Electronic Publication: 2023 May 09.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*/metabolism
Humans ; Motor Neurons/metabolism ; Mutation ; Mitochondria/metabolism ; Adenosine Triphosphate/metabolism ; RNA-Binding Protein FUS/genetics ; RNA-Binding Protein FUS/metabolism ; RNA-Binding Protein FUS/pharmacology
Czasopismo naukowe
Tytuł:
Early Alterations in Structural and Functional Properties in the Neuromuscular Junctions of Mutant FUS Mice.
Autorzy:
Mukhamedyarov MA; Department of Normal Physiology, Kazan State Medial University, 49 Butlerova St., Kazan 420012, Russia.
Khabibrakhmanov AN; Department of Normal Physiology, Kazan State Medial University, 49 Butlerova St., Kazan 420012, Russia.
Khuzakhmetova VF; Kazan Institute of Biochemistry and Biophysics, Federal Research Center ''Kazan Scientific Center of RAS', 2/31 Lobachevsky St., P.O. Box 30, Kazan 420111, Russia.
Giniatullin AR; Department of Normal Physiology, Kazan State Medial University, 49 Butlerova St., Kazan 420012, Russia.; Kazan Institute of Biochemistry and Biophysics, Federal Research Center ''Kazan Scientific Center of RAS', 2/31 Lobachevsky St., P.O. Box 30, Kazan 420111, Russia.
Zakirjanova GF; Department of Normal Physiology, Kazan State Medial University, 49 Butlerova St., Kazan 420012, Russia.; Kazan Institute of Biochemistry and Biophysics, Federal Research Center ''Kazan Scientific Center of RAS', 2/31 Lobachevsky St., P.O. Box 30, Kazan 420111, Russia.
Zhilyakov NV; Kazan Institute of Biochemistry and Biophysics, Federal Research Center ''Kazan Scientific Center of RAS', 2/31 Lobachevsky St., P.O. Box 30, Kazan 420111, Russia.
Mukhutdinova KA; Department of Normal Physiology, Kazan State Medial University, 49 Butlerova St., Kazan 420012, Russia.
Samigullin DV; Kazan Institute of Biochemistry and Biophysics, Federal Research Center ''Kazan Scientific Center of RAS', 2/31 Lobachevsky St., P.O. Box 30, Kazan 420111, Russia.; Department of Radiophotonics and Microwave Technologies, Kazan National Research Technical University, 10 K. Marx St., Kazan 420111, Russia.
Grigoryev PN; Department of Normal Physiology, Kazan State Medial University, 49 Butlerova St., Kazan 420012, Russia.
Zakharov AV; Department of Normal Physiology, Kazan State Medial University, 49 Butlerova St., Kazan 420012, Russia.; Laboratory of Neurobiology, Kazan Federal University, Kazan 420008, Russia.
Zefirov AL; Department of Normal Physiology, Kazan State Medial University, 49 Butlerova St., Kazan 420012, Russia.
Petrov AM; Department of Normal Physiology, Kazan State Medial University, 49 Butlerova St., Kazan 420012, Russia.; Kazan Institute of Biochemistry and Biophysics, Federal Research Center ''Kazan Scientific Center of RAS', 2/31 Lobachevsky St., P.O. Box 30, Kazan 420111, Russia.
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Źródło:
International journal of molecular sciences [Int J Mol Sci] 2023 May 19; Vol. 24 (10). Date of Electronic Publication: 2023 May 19.
Typ publikacji:
Journal Article
MeSH Terms:
Amyotrophic Lateral Sclerosis*/genetics
Animals ; Mice ; RNA-Binding Protein FUS/genetics ; Synapsins/genetics ; Synapsins/metabolism ; Neuromuscular Junction/metabolism ; Neurotransmitter Agents/metabolism
Czasopismo naukowe
Tytuł:
Accuracy of a machine learning method based on structural and locational information from AlphaFold2 for predicting the pathogenicity of TARDBP and FUS gene variants in ALS.
Autorzy:
Hatano Y; Department of Neurology, Brain Research Institute, Niigata University, 1-757 Asahimachidori, Chuo-ku, Niigata-shi, Niigata, 951-8585, Japan.
Ishihara T; Department of Neurology, Brain Research Institute, Niigata University, 1-757 Asahimachidori, Chuo-ku, Niigata-shi, Niigata, 951-8585, Japan. .
Onodera O; Department of Neurology, Brain Research Institute, Niigata University, 1-757 Asahimachidori, Chuo-ku, Niigata-shi, Niigata, 951-8585, Japan.
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Źródło:
BMC bioinformatics [BMC Bioinformatics] 2023 May 19; Vol. 24 (1), pp. 206. Date of Electronic Publication: 2023 May 19.
Typ publikacji:
Journal Article
MeSH Terms:
Amyotrophic Lateral Sclerosis*/genetics
Humans ; Superoxide Dismutase-1/genetics ; Virulence ; Mutation ; Mutation, Missense ; Autophagy-Related Proteins/genetics ; Adaptor Proteins, Signal Transducing/genetics ; DNA Helicases/genetics ; RNA Helicases ; Multifunctional Enzymes/genetics ; RNA-Binding Protein FUS/genetics
Czasopismo naukowe
Tytuł:
FUS fibrillation occurs through a nucleation-based process below the critical concentration required for liquid-liquid phase separation.
Autorzy:
Bertrand E; SABNP, Univ Evry, INSERM U1204, Université Paris-Saclay, 91025, Evry, France.
Demongin C; SABNP, Univ Evry, INSERM U1204, Université Paris-Saclay, 91025, Evry, France.
Dobra I; SABNP, Univ Evry, INSERM U1204, Université Paris-Saclay, 91025, Evry, France.
Rengifo-Gonzalez JC; SABNP, Univ Evry, INSERM U1204, Université Paris-Saclay, 91025, Evry, France.
Singatulina AS; SABNP, Univ Evry, INSERM U1204, Université Paris-Saclay, 91025, Evry, France.; Institute of Chemical Biology and Fundamental Medicine, Novosibirsk, Russia, 630090.
Sukhanova MV; Institute of Chemical Biology and Fundamental Medicine, Novosibirsk, Russia, 630090.
Lavrik OI; Institute of Chemical Biology and Fundamental Medicine, Novosibirsk, Russia, 630090.
Pastré D; SABNP, Univ Evry, INSERM U1204, Université Paris-Saclay, 91025, Evry, France.
Hamon L; SABNP, Univ Evry, INSERM U1204, Université Paris-Saclay, 91025, Evry, France. .
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Źródło:
Scientific reports [Sci Rep] 2023 May 13; Vol. 13 (1), pp. 7772. Date of Electronic Publication: 2023 May 13.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Prions*/metabolism
Neurodegenerative Diseases*/metabolism
Amyotrophic Lateral Sclerosis*/metabolism
Humans ; RNA, Messenger/metabolism ; Cytoplasm/metabolism ; Phosphorylation ; RNA-Binding Protein FUS/metabolism
Czasopismo naukowe
Tytuł:
Nuclear RNA transcript levels modulate nucleocytoplasmic distribution of ALS/FTD-associated protein FUS.
Autorzy:
Tsai YL; Department of Biological Sciences, Columbia University, New York, NY, 10027, USA.
Mu YC; Department of Biological Sciences, Columbia University, New York, NY, 10027, USA.
Manley JL; Department of Biological Sciences, Columbia University, New York, NY, 10027, USA. .
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Źródło:
Scientific reports [Sci Rep] 2022 May 17; Vol. 12 (1), pp. 8180. Date of Electronic Publication: 2022 May 17.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural
MeSH Terms:
Amyotrophic Lateral Sclerosis*/genetics
Amyotrophic Lateral Sclerosis*/metabolism
Frontotemporal Dementia*/genetics
Frontotemporal Dementia*/metabolism
RNA-Binding Protein FUS*/genetics
RNA-Binding Protein FUS*/metabolism
Cytoplasm/metabolism ; Humans ; Mutation ; RNA, Nuclear/genetics ; RNA, Nuclear/metabolism
Czasopismo naukowe
Tytuł:
FUS regulates a subset of snoRNA expression and modulates the level of rRNA modifications.
Autorzy:
Gawade K; Laboratory of RNA Processing, Department of Gene Expression, Institute of Molecular Biology and Biotechnology, Faculty of Biology, Adam Mickiewicz University in Poznan, 61-614, Poznan, Poland.; Center for Advanced Technology, Adam Mickiewicz University in Poznan, 61-614, Poznan, Poland.
Plewka P; Laboratory of RNA Processing, Department of Gene Expression, Institute of Molecular Biology and Biotechnology, Faculty of Biology, Adam Mickiewicz University in Poznan, 61-614, Poznan, Poland.; Center for Advanced Technology, Adam Mickiewicz University in Poznan, 61-614, Poznan, Poland.
Häfner SJ; Biotech Research and Innovation Centre, University of Copenhagen, 2200, Copenhagen, Denmark.
Lund AH; Biotech Research and Innovation Centre, University of Copenhagen, 2200, Copenhagen, Denmark.
Marchand V; Université de Lorraine, UAR2008/US40 IBSLor CNRS-INSERM and UMR7365 IMoPA CNRS, Nancy, France.
Motorin Y; Université de Lorraine, UAR2008/US40 IBSLor CNRS-INSERM and UMR7365 IMoPA CNRS, Nancy, France.
Szczesniak MW; Institute of Human Biology and Evolution, Faculty of Biology, Adam Mickiewicz University in Poznan, 61-614, Poznan, Poland.
Raczynska KD; Laboratory of RNA Processing, Department of Gene Expression, Institute of Molecular Biology and Biotechnology, Faculty of Biology, Adam Mickiewicz University in Poznan, 61-614, Poznan, Poland. .; Center for Advanced Technology, Adam Mickiewicz University in Poznan, 61-614, Poznan, Poland. .
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Źródło:
Scientific reports [Sci Rep] 2023 Feb 20; Vol. 13 (1), pp. 2974. Date of Electronic Publication: 2023 Feb 20.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*
Neuroblastoma*
Humans ; RNA, Small Nucleolar/genetics ; HEK293 Cells ; RNA, Ribosomal/genetics ; RNA-Binding Protein FUS/genetics
Czasopismo naukowe
Tytuł:
FUS Alters circRNA Metabolism in Human Motor Neurons Carrying the ALS-Linked P525L Mutation.
Autorzy:
Colantoni A; Department of Biology and Biotechnologies 'Charles Darwin', Sapienza University of Rome, 00185 Rome, Italy.; Center for Life Nano- & Neuro-Science, Fondazione Istituto Italiano di Tecnologia (IIT), 00161 Rome, Italy.
Capauto D; Department of Biology and Biotechnologies 'Charles Darwin', Sapienza University of Rome, 00185 Rome, Italy.
Alfano V; Department of Biology and Biotechnologies 'Charles Darwin', Sapienza University of Rome, 00185 Rome, Italy.
D'Ambra E; Center for Life Nano- & Neuro-Science, Fondazione Istituto Italiano di Tecnologia (IIT), 00161 Rome, Italy.
D'Uva S; Department of Biology and Biotechnologies 'Charles Darwin', Sapienza University of Rome, 00185 Rome, Italy.
Tartaglia GG; Department of Biology and Biotechnologies 'Charles Darwin', Sapienza University of Rome, 00185 Rome, Italy.; Centre for Human Technologies (CHT), Istituto Italiano di Tecnologia (IIT), 16152 Genova, Italy.
Morlando M; Department of Pharmaceutical Sciences, Department of Excellence 2018-2022, University of Perugia, 06123 Perugia, Italy.
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Źródło:
International journal of molecular sciences [Int J Mol Sci] 2023 Feb 06; Vol. 24 (4). Date of Electronic Publication: 2023 Feb 06.
Typ publikacji:
Journal Article
MeSH Terms:
Amyotrophic Lateral Sclerosis*/metabolism
MicroRNAs*/metabolism
Humans ; RNA, Circular/metabolism ; Motor Neurons/metabolism ; Mutation ; RNA-Binding Protein FUS/genetics
Czasopismo naukowe
Tytuł:
FUS-ALS hiPSC-derived astrocytes impair human motor units through both gain-of-toxicity and loss-of-support mechanisms.
Autorzy:
Stoklund Dittlau K; Department of Neurosciences, Experimental Neurology and Leuven Brain Institute, KU Leuven - University of Leuven, 3000, Leuven, Belgium.; VIB Center for Brain & Disease Research, Laboratory of Neurobiology, 3000, Leuven, Belgium.
Terrie L; Department of Development and Regeneration, KU Leuven - University of Leuven, Campus Kulak, 8500, Kortrijk, Belgium.
Baatsen P; KU Leuven - University of Leuven, EM-Platform of the VIB Bio Imaging Core and VIB Center for Brain and Disease Research, Research Group Molecular Neurobiology, 3000, Leuven, Belgium.
Kerstens A; KU Leuven - University of Leuven, VIB Bio Imaging Core; VIB Center for Brain & Disease Research, 3000, Leuven, Belgium.
De Swert L; VIB Nucleomics Core, VIB, Herestraat 49, 3000, Leuven, Belgium.
Janky R; VIB Nucleomics Core, VIB, Herestraat 49, 3000, Leuven, Belgium.
Corthout N; KU Leuven - University of Leuven, VIB Bio Imaging Core; VIB Center for Brain & Disease Research, 3000, Leuven, Belgium.
Masrori P; Department of Neurosciences, Experimental Neurology and Leuven Brain Institute, KU Leuven - University of Leuven, 3000, Leuven, Belgium.; VIB Center for Brain & Disease Research, Laboratory of Neurobiology, 3000, Leuven, Belgium.; Department of Neurology, University Hospitals Leuven, 3000, Leuven, Belgium.
Van Damme P; Department of Neurosciences, Experimental Neurology and Leuven Brain Institute, KU Leuven - University of Leuven, 3000, Leuven, Belgium.; VIB Center for Brain & Disease Research, Laboratory of Neurobiology, 3000, Leuven, Belgium.; Department of Neurology, University Hospitals Leuven, 3000, Leuven, Belgium.
Hyttel P; Department of Veterinary and Animal Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, 1870, Frederiksberg C, Denmark.
Meyer M; Department of Neurology, Odense University Hospital, 5000, Odense, Denmark.; Department of Neurobiology Research, Institute of Molecular Medicine, University of Southern Denmark, 5000, Odense, Denmark.
Thorrez L; Department of Development and Regeneration, KU Leuven - University of Leuven, Campus Kulak, 8500, Kortrijk, Belgium.
Freude K; Department of Veterinary and Animal Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, 1870, Frederiksberg C, Denmark.
Van Den Bosch L; Department of Neurosciences, Experimental Neurology and Leuven Brain Institute, KU Leuven - University of Leuven, 3000, Leuven, Belgium. .; VIB Center for Brain & Disease Research, Laboratory of Neurobiology, 3000, Leuven, Belgium. .
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Źródło:
Molecular neurodegeneration [Mol Neurodegener] 2023 Jan 18; Vol. 18 (1), pp. 5. Date of Electronic Publication: 2023 Jan 18.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*/metabolism
Induced Pluripotent Stem Cells*/metabolism
Humans ; Astrocytes/metabolism ; Motor Neurons/metabolism ; Neuromuscular Junction ; RNA-Binding Protein FUS/physiology
Czasopismo naukowe
Tytuł:
FUS mutations in Asian amyotrophic lateral sclerosis patients: a case report and literature review of genotype-phenotype correlations.
Autorzy:
Lu T; Department of Neurology, The First Hospital of Wuhan, Wuhan, PR China.; The First Clinical Medical Institute, Hubei University of Traditional Chinese Medicine, Wuhan, PR China.
Yang J; Department of Neurology, The First Hospital of Wuhan, Wuhan, PR China.
Luo L; Department of Neurology, The First Hospital of Wuhan, Wuhan, PR China.
Wei D; Department of Neurology, The First Hospital of Wuhan, Wuhan, PR China.
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Źródło:
Amyotrophic lateral sclerosis & frontotemporal degeneration [Amyotroph Lateral Scler Frontotemporal Degener] 2022 Nov; Vol. 23 (7-8), pp. 580-584. Date of Electronic Publication: 2022 Mar 01.
Typ publikacji:
Case Reports; Journal Article; Review; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*/diagnosis
Amyotrophic Lateral Sclerosis*/genetics
Amyotrophic Lateral Sclerosis*/pathology
Neurodegenerative Diseases*
Humans ; RNA-Binding Protein FUS/genetics ; Genetic Association Studies ; Mutation/genetics
Czasopismo naukowe
Tytuł:
Wild-type FUS corrects ALS-like disease induced by cytoplasmic mutant FUS through autoregulation.
Autorzy:
Sanjuan-Ruiz I; Mécanismes centraux et périphériques de la neurodégénérescence, Centre de Recherches en Biomédecine, Université de Strasbourg, Inserm, UMR-S1118, Strasbourg, France.
Govea-Perez N; Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, USA.
McAlonis-Downes M; Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, USA.
Dieterle S; Mécanismes centraux et périphériques de la neurodégénérescence, Centre de Recherches en Biomédecine, Université de Strasbourg, Inserm, UMR-S1118, Strasbourg, France.
Megat S; Mécanismes centraux et périphériques de la neurodégénérescence, Centre de Recherches en Biomédecine, Université de Strasbourg, Inserm, UMR-S1118, Strasbourg, France.
Dirrig-Grosch S; Mécanismes centraux et périphériques de la neurodégénérescence, Centre de Recherches en Biomédecine, Université de Strasbourg, Inserm, UMR-S1118, Strasbourg, France.
Picchiarelli G; Mécanismes centraux et périphériques de la neurodégénérescence, Centre de Recherches en Biomédecine, Université de Strasbourg, Inserm, UMR-S1118, Strasbourg, France.
Piol D; VIB-KU Leuven Center for Brain and Disease Research, Department of Neurosciences, KU Leuven, Leuven, Belgium.
Zhu Q; Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, USA.
Myers B; Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, USA.
Lee CZ; Department of Neurology, Massachusetts General Hospital, The Sean M. Healey and AMG Center for ALS at Mass General, Harvard Medical School, Boston, MA, USA.; Broad Institute of Harvard University and MIT, Cambridge, MA, USA.
Cleveland DW; Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, USA.
Lagier-Tourenne C; Department of Neurology, Massachusetts General Hospital, The Sean M. Healey and AMG Center for ALS at Mass General, Harvard Medical School, Boston, MA, USA.; Broad Institute of Harvard University and MIT, Cambridge, MA, USA.
Da Cruz S; Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, USA. .; VIB-KU Leuven Center for Brain and Disease Research, Department of Neurosciences, KU Leuven, Leuven, Belgium. .
Dupuis L; Mécanismes centraux et périphériques de la neurodégénérescence, Centre de Recherches en Biomédecine, Université de Strasbourg, Inserm, UMR-S1118, Strasbourg, France. .
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Źródło:
Molecular neurodegeneration [Mol Neurodegener] 2021 Sep 06; Vol. 16 (1), pp. 61. Date of Electronic Publication: 2021 Sep 06.
Typ publikacji:
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms:
Disease Models, Animal*
Amyotrophic Lateral Sclerosis/*therapy
Gene Expression Regulation/*genetics
RNA-Binding Protein FUS/*physiology
Alleles ; Amyotrophic Lateral Sclerosis/genetics ; Animals ; Cytoplasm/metabolism ; Frontotemporal Dementia/genetics ; Genes, Lethal ; Genetic Complementation Test ; Humans ; Introns/genetics ; Mice ; Mice, Transgenic ; Mutation ; Protein Binding ; RNA Precursors/metabolism ; RNA-Binding Protein FUS/deficiency ; RNA-Binding Protein FUS/genetics ; Recombinant Proteins/metabolism ; Transgenes
Czasopismo naukowe
Tytuł:
Conformational change of RNA-helicase DHX30 by ALS/FTD-linked FUS induces mitochondrial dysfunction and cytosolic aggregates.
Autorzy:
Hikiami R; Department of Neurology, Shiga University of Medical Science, Seta-Tsukinowa-Cho, Otsu, Shiga, 520-2192, Japan.; Molecular Neuroscience Research Center, Shiga University of Medical Science, Otsu, Shiga, 520-2192, Japan.; Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, 606-8501, Japan.
Morimura T; Research Center for Animal Life Science, Shiga University of Medical Science, Otsu, Shiga, 520-2192, Japan.
Ayaki T; Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, 606-8501, Japan.
Tsukiyama T; Research Center for Animal Life Science, Shiga University of Medical Science, Otsu, Shiga, 520-2192, Japan.; Institute for the Advanced Study of Human Biology (WPI-ASHBi), Kyoto University, Kyoto, 606-8501, Japan.
Morimura N; Department of Integrative Physiology, Shiga University of Medical Science, Otsu, Shiga, 520-2192, Japan.
Kusui M; Department of Neurology, Shiga University of Medical Science, Seta-Tsukinowa-Cho, Otsu, Shiga, 520-2192, Japan.
Wada H; Department of Neurology, Shiga University of Medical Science, Seta-Tsukinowa-Cho, Otsu, Shiga, 520-2192, Japan.
Minamiyama S; Department of Neurology, Shiga University of Medical Science, Seta-Tsukinowa-Cho, Otsu, Shiga, 520-2192, Japan.; Molecular Neuroscience Research Center, Shiga University of Medical Science, Otsu, Shiga, 520-2192, Japan.; Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, 606-8501, Japan.
Shodai A; Department of Neurology, Shiga University of Medical Science, Seta-Tsukinowa-Cho, Otsu, Shiga, 520-2192, Japan.
Asada-Utsugi M; Department of Neurology, Shiga University of Medical Science, Seta-Tsukinowa-Cho, Otsu, Shiga, 520-2192, Japan.
Muramatsu SI; Division of Neurological Gene Therapy, Center for Open Innovation, Jichi Medical University, Tochigi, 320-0498, Japan.; Center for Gene and Cell Therapy, The Institute of Medical Science, The University of Tokyo, Tokyo, 108-0071, Japan.
Ueki T; Department of Integrative Anatomy, Graduate School of Medical Sciences, Nagoya City University, Nagoya, 467-8601, Japan.
Takahashi R; Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto, 606-8501, Japan.
Urushitani M; Department of Neurology, Shiga University of Medical Science, Seta-Tsukinowa-Cho, Otsu, Shiga, 520-2192, Japan. .; Molecular Neuroscience Research Center, Shiga University of Medical Science, Otsu, Shiga, 520-2192, Japan. .
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Źródło:
Scientific reports [Sci Rep] 2022 Sep 26; Vol. 12 (1), pp. 16030. Date of Electronic Publication: 2022 Sep 26.
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*/genetics
Frontotemporal Dementia*
Detergents ; Disulfides ; Humans ; Mitochondria/genetics ; Mutation ; RNA ; RNA Helicases/genetics ; RNA-Binding Protein FUS/chemistry ; RNA-Binding Protein FUS/genetics
Czasopismo naukowe
Tytuł:
Molecular dynamics and protein frustration analysis of human fused in Sarcoma protein variants in Amyotrophic Lateral Sclerosis type 6: An In Silico approach.
Autorzy:
Bonet LFS; Department of Genetics and Molecular Biology, Laboratory of Bioinformatics and Computational Biology, Federal University of the State of Rio de Janeiro, Rio de Janeiro, Brazil.
Loureiro JP; Department of Genetics and Molecular Biology, Laboratory of Bioinformatics and Computational Biology, Federal University of the State of Rio de Janeiro, Rio de Janeiro, Brazil.
Pereira GRC; Department of Genetics and Molecular Biology, Laboratory of Bioinformatics and Computational Biology, Federal University of the State of Rio de Janeiro, Rio de Janeiro, Brazil.
Da Silva ANR; Department of Genetics and Molecular Biology, Laboratory of Bioinformatics and Computational Biology, Federal University of the State of Rio de Janeiro, Rio de Janeiro, Brazil.
De Mesquita JF; Department of Genetics and Molecular Biology, Laboratory of Bioinformatics and Computational Biology, Federal University of the State of Rio de Janeiro, Rio de Janeiro, Brazil.
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Źródło:
PloS one [PLoS One] 2021 Sep 29; Vol. 16 (9), pp. e0258061. Date of Electronic Publication: 2021 Sep 29 (Print Publication: 2021).
Typ publikacji:
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms:
Computer Simulation*
Mutation*
Amyotrophic Lateral Sclerosis/*genetics
RNA-Binding Protein FUS/*genetics
Amyotrophic Lateral Sclerosis/mortality ; DNA Mutational Analysis ; Databases, Protein ; Molecular Dynamics Simulation ; Polymorphism, Single Nucleotide ; RNA-Binding Protein FUS/metabolism
Czasopismo naukowe
Tytuł:
Gene Therapy in Amyotrophic Lateral Sclerosis.
Autorzy:
Fang T; Department of Neurology, University of Massachusetts Chan Medical School, Worcester, MA 01655, USA.
Je G; Department of Neurology, University of Massachusetts Chan Medical School, Worcester, MA 01655, USA.
Pacut P; Department of Neurology, University of Massachusetts Chan Medical School, Worcester, MA 01655, USA.
Keyhanian K; Department of Neurology, University of Massachusetts Chan Medical School, Worcester, MA 01655, USA.
Gao J; Department of Neurology, University of Massachusetts Chan Medical School, Worcester, MA 01655, USA.
Ghasemi M; Department of Neurology, University of Massachusetts Chan Medical School, Worcester, MA 01655, USA.
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Źródło:
Cells [Cells] 2022 Jun 29; Vol. 11 (13). Date of Electronic Publication: 2022 Jun 29.
Typ publikacji:
Journal Article; Review; Research Support, N.I.H., Extramural
MeSH Terms:
Amyotrophic Lateral Sclerosis*/genetics
Amyotrophic Lateral Sclerosis*/metabolism
Amyotrophic Lateral Sclerosis*/therapy
Frontotemporal Dementia*/genetics
C9orf72 Protein/genetics ; C9orf72 Protein/metabolism ; Genetic Therapy ; Humans ; RNA-Binding Protein FUS/genetics ; RNA-Binding Protein FUS/metabolism ; Superoxide Dismutase/metabolism ; Superoxide Dismutase-1/genetics ; Superoxide Dismutase-1/metabolism
Czasopismo naukowe
Tytuł:
Broadening the clinical spectrum of FUS mutations: a case with monomelic amyotrophy with a late progression to amyotrophic lateral sclerosis.
Autorzy:
Moglia C; ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy. .; Struttura Complessa Neurologia IU, Azienda Ospedaliero Universitaria Città della Salute e della Scienza of Turin, via Cherasco 15, 10126, Turin, Italy. .
Calvo A; ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Struttura Complessa Neurologia IU, Azienda Ospedaliero Universitaria Città della Salute e della Scienza of Turin, via Cherasco 15, 10126, Turin, Italy.
Brunetti M; ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Struttura Complessa Neurologia IU, Azienda Ospedaliero Universitaria Città della Salute e della Scienza of Turin, via Cherasco 15, 10126, Turin, Italy.
Chiò A; ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Struttura Complessa Neurologia IU, Azienda Ospedaliero Universitaria Città della Salute e della Scienza of Turin, via Cherasco 15, 10126, Turin, Italy.; Neuroscience Institute of Torino, Turin, Italy.
Grassano M; ALS Center, 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.
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Źródło:
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology [Neurol Sci] 2021 Mar; Vol. 42 (3), pp. 1207-1209. Date of Electronic Publication: 2020 Oct 01.
Typ publikacji:
Case Reports; Letter; Research Support, Non-U.S. Gov't
MeSH Terms:
Amyotrophic Lateral Sclerosis*/complications
Amyotrophic Lateral Sclerosis*/genetics
RNA-Binding Protein FUS*/genetics
Spinal Muscular Atrophies of Childhood*
Humans ; Mutation
SCR Disease Name:
Amyotrophy, monomelic
Raport

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