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Mitochondrial and energy metabolism dysfunctions are hallmarks of TDP-43G376D fibroblasts from members of an Amyotrophic Lateral Sclerosis family.

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Title: Mitochondrial and energy metabolism dysfunctions are hallmarks of TDP-43G376D fibroblasts from members of an Amyotrophic Lateral Sclerosis family.
Authors: Perciballi E; Institute for Stem-Cell Biology, Regenerative Medicine and Innovative Therapies (ISBReMIT), Production Unit of Advanced Therapies (UPTA), Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy.; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy., Bovio F; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy., Ferro S; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy., Forcella M; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy., Rosati J; Cellular Reprogramming Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy.; Saint Camillus International, University of Health Sciences, Rome, Italy., Carletti RM; Institute for Stem-Cell Biology, Regenerative Medicine and Innovative Therapies (ISBReMIT), Production Unit of Advanced Therapies (UPTA), Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy., D'Anzi A; Cellular Reprogramming Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy., Gelati M; Institute for Stem-Cell Biology, Regenerative Medicine and Innovative Therapies (ISBReMIT), Production Unit of Advanced Therapies (UPTA), Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy., La Bella V; ALS Clinical Research Centre and Laboratory of Neurochemistry, Department of Experimental Biomedicine and Clinical Neurosciences, University of Palermo, Via del Vespro, 129, 90127, Palermo, Italy., Innocenti M; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy., Spataro R; ALS Clinical Research Centre and Laboratory of Neurochemistry, Department of Experimental Biomedicine and Clinical Neurosciences, University of Palermo, Via del Vespro, 129, 90127, Palermo, Italy.; Intensive Neurorehabilitation Unit, Villa delle Ginestre Hospital, Via Castellana, 145 - 90135, Palermo, Italy., Pecoraro M; ALS Clinical Research Centre and Laboratory of Neurochemistry, Department of Experimental Biomedicine and Clinical Neurosciences, University of Palermo, Via del Vespro, 129, 90127, Palermo, Italy., Lombardi I; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy., Vulcano E; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy., Ruotolo G; Cellular Reprogramming Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy., Mercurio S; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy., Sabatelli M; Adult NEMO Clinical Center, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli 8, 00168, Rome, Italy.; Section of Neurology, Università Cattolica del Sacro Cuore, Largo Francesco Vito, 1, 00168, Rome, Italy., Lattante S; Unit of Medical Genetics, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli 8, 00168, Rome, Italy., Malm T; A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Neulaniementie, 2, FI-70211, Kuopio, Finland., Ohtonen S; A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Neulaniementie, 2, FI-70211, Kuopio, Finland., Vescovi AL; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.; Abu Dhabi Stem Cells Center, Abu Dhabi, United Arab Emirates., Fusi P; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy. paola.fusi@unimib.it., Ferrari D; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy. daniela.ferrari@unimib.it.
Source: Cell death & disease [Cell Death Dis] 2025 Apr 10; Vol. 16 (1), pp. 272. Date of Electronic Publication: 2025 Apr 10.
Publication Type: Journal Article
Journal Info: Publisher: Nature Pub. Group Country of Publication: England NLM ID: 101524092 Publication Model: Electronic Cited Medium: Internet ISSN: 2041-4889 (Electronic) NLM ISO Abbreviation: Cell Death Dis Subsets: MEDLINE
Database: MEDLINE Ultimate
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  Data: Mitochondrial and energy metabolism dysfunctions are hallmarks of TDP-43<superscript>G376D</superscript> fibroblasts from members of an Amyotrophic Lateral Sclerosis family.
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  Data: <searchLink fieldCode="AU" term="%22Perciballi+E%22">Perciballi E</searchLink>; Institute for Stem-Cell Biology, Regenerative Medicine and Innovative Therapies (ISBReMIT), Production Unit of Advanced Therapies (UPTA), Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy.; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Bovio+F%22">Bovio F</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Ferro+S%22">Ferro S</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Forcella+M%22">Forcella M</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Rosati+J%22">Rosati J</searchLink>; Cellular Reprogramming Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy.; Saint Camillus International, University of Health Sciences, Rome, Italy.<br /><searchLink fieldCode="AU" term="%22Carletti+RM%22">Carletti RM</searchLink>; Institute for Stem-Cell Biology, Regenerative Medicine and Innovative Therapies (ISBReMIT), Production Unit of Advanced Therapies (UPTA), Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy.<br /><searchLink fieldCode="AU" term="%22D'Anzi+A%22">D'Anzi A</searchLink>; Cellular Reprogramming Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy.<br /><searchLink fieldCode="AU" term="%22Gelati+M%22">Gelati M</searchLink>; Institute for Stem-Cell Biology, Regenerative Medicine and Innovative Therapies (ISBReMIT), Production Unit of Advanced Therapies (UPTA), Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy.<br /><searchLink fieldCode="AU" term="%22La+Bella+V%22">La Bella V</searchLink>; ALS Clinical Research Centre and Laboratory of Neurochemistry, Department of Experimental Biomedicine and Clinical Neurosciences, University of Palermo, Via del Vespro, 129, 90127, Palermo, Italy.<br /><searchLink fieldCode="AU" term="%22Innocenti+M%22">Innocenti M</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Spataro+R%22">Spataro R</searchLink>; ALS Clinical Research Centre and Laboratory of Neurochemistry, Department of Experimental Biomedicine and Clinical Neurosciences, University of Palermo, Via del Vespro, 129, 90127, Palermo, Italy.; Intensive Neurorehabilitation Unit, Villa delle Ginestre Hospital, Via Castellana, 145 - 90135, Palermo, Italy.<br /><searchLink fieldCode="AU" term="%22Pecoraro+M%22">Pecoraro M</searchLink>; ALS Clinical Research Centre and Laboratory of Neurochemistry, Department of Experimental Biomedicine and Clinical Neurosciences, University of Palermo, Via del Vespro, 129, 90127, Palermo, Italy.<br /><searchLink fieldCode="AU" term="%22Lombardi+I%22">Lombardi I</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Vulcano+E%22">Vulcano E</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Ruotolo+G%22">Ruotolo G</searchLink>; Cellular Reprogramming Unit, Fondazione IRCCS Casa Sollievo della Sofferenza, Viale dei Cappuccini 1, 71013, San Giovanni Rotondo, Italy.<br /><searchLink fieldCode="AU" term="%22Mercurio+S%22">Mercurio S</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Sabatelli+M%22">Sabatelli M</searchLink>; Adult NEMO Clinical Center, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli 8, 00168, Rome, Italy.; Section of Neurology, Università Cattolica del Sacro Cuore, Largo Francesco Vito, 1, 00168, Rome, Italy.<br /><searchLink fieldCode="AU" term="%22Lattante+S%22">Lattante S</searchLink>; Unit of Medical Genetics, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli 8, 00168, Rome, Italy.<br /><searchLink fieldCode="AU" term="%22Malm+T%22">Malm T</searchLink>; A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Neulaniementie, 2, FI-70211, Kuopio, Finland.<br /><searchLink fieldCode="AU" term="%22Ohtonen+S%22">Ohtonen S</searchLink>; A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Neulaniementie, 2, FI-70211, Kuopio, Finland.<br /><searchLink fieldCode="AU" term="%22Vescovi+AL%22">Vescovi AL</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy.; Abu Dhabi Stem Cells Center, Abu Dhabi, United Arab Emirates.<br /><searchLink fieldCode="AU" term="%22Fusi+P%22">Fusi P</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy. paola.fusi@unimib.it.<br /><searchLink fieldCode="AU" term="%22Ferrari+D%22">Ferrari D</searchLink>; Department of Biotechnology and Biosciences, University of Milano-Bicocca, P.zza della Scienza, 2, 20126, Milan, Italy. daniela.ferrari@unimib.it.
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