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Mitochondrial dysfunction in fibroblasts of Multiple System Atrophy |
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| Authors: | Giacomo Monzio Compagnoni Giulio Kleiner Andreina Bordoni Francesco Fortunato Dario Ronchi Sabrina Salani Marianna Guida Corrado Corti Irene Pichler Christian Bergamini Romana Fato Maria Teresa Pellecchia Annamaria Vallelunga Francesca Del Sorbo Antonio Elia Chiara Reale Barbara Garavaglia Gabriele Mora Alessio Di Fonzo |
| Affiliation: | 1. IRCCS Foundation Ca'' Granda Ospedale Maggiore Policlinico, Dino Ferrari Center, Neuroscience Section, Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy;2. Department of Neurology, Columbia University, New York 10032, NY, USA;3. Institute for Biomedicine, Eurac Research, Via Galvani 31, 39100 Bolzano, Italy;4. Department of Pharmacy and Biotechnology (FaBiT), University of Bologna, Bologna, Italy;5. Neuroscience Section, Department of Medicine, Surgery and Dentistry “Scuola Medica Salernitana”, University of Salerno, Italy;6. Neurology Unit I, Neurological Institute “C. Besta” IRCCS Foundation, Milan, Italy;7. Medical Genetics and Neurogenetics Unit, IRCCS Foundation Istituto Neurologico Carlo Besta, Milan, Italy;8. Department of Neurological Rehabilitation, ICS Maugeri, IRCCS, Istituto Scientifico di Milano, Milan, Italy;9. Department of Neurology, Humanitas Research Hospital, Rozzano, Milan, Italy;10. U.O. Neurofisiopatologia, IRCCS Foundation Ca'' Granda Ospedale Maggiore Policlinico, Milan, Italy |
| Abstract: | Multiple System Atrophy is a severe neurodegenerative disorder which is characterized by a variable clinical presentation and a broad neuropathological spectrum. The pathogenic mechanisms are almost completely unknown. In the present study, we established a cellular model of MSA by using fibroblasts' primary cultures and performed several experiments to investigate the causative mechanisms of the disease, with a particular focus on mitochondrial functioning.Fibroblasts' analyses (7 MSA-P, 7 MSA-C and 6 healthy controls) displayed several anomalies in patients: an impairment of respiratory chain activity, in particular for succinate Coenzyme Q reductase (p? |
| Keywords: | Multiple System Atrophy Cellular models Fibroblasts Mitochondria |
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