Mitochondrial Dysregulation and Impaired Autophagy in iPSC-Derived Dopaminergic Neurons of Multiple System Atrophy
Articolo
Data di Pubblicazione:
2018
Citazione:
Mitochondrial Dysregulation and Impaired Autophagy in iPSC-Derived Dopaminergic Neurons of Multiple System Atrophy / G. Monzio Compagnoni, G. Kleiner, M. Samarani, M. Aureli, G. Faustini, A. Bellucci, D. Ronchi, A. Bordoni, M. Garbellini, S. Salani, F. Fortunato, E. Frattini, E. Abati, C. Bergamini, R. Fato, S. Tabano, M. Miozzo, G. Serratto, M. Passafaro, M. Deleidi, R. Silipigni, M. Nizzardo, N. Bresolin, G. Comi, S. Corti, C. Quinzii, A. Di Fonzo. - In: STEM CELL REPORTS. - ISSN 2213-6711. - 11:5(2018 Nov 13), pp. 1185-1198. [10.1016/j.stemcr.2018.09.007]
Abstract:
Multiple system atrophy (MSA) is a progressive neurodegenerative disease that affects several areas of the CNS, whose pathogenesis is still widely unclear and for which an effective treatment is lacking. We have generated induced pluripotent stem cell-derived dopaminergic neurons from four MSA patients and four healthy controls and from two monozygotic twins discordant for the disease. In this model, we have demonstrated an aberrant autophagic flow and a mitochondrial dysregulation involving respiratory chain activity, mitochondrial content, and CoQ10 biosynthesis. These defective mechanisms may contribute to the onset of the disease, representing potential therapeutic targets. Monzio Compagnoni et al. present an iPSC-based neuronal in vitro model of multiple system atrophy. Patients' dopaminergic neurons display a dysregulation of mitochondrial functioning and autophagy, suggesting new hints for the comprehension of the pathogenesis of the disease.
Tipologia IRIS:
01 - Articolo su periodico
Elenco autori:
G. Monzio Compagnoni, G. Kleiner, M. Samarani, M. Aureli, G. Faustini, A. Bellucci, D. Ronchi, A. Bordoni, M. Garbellini, S. Salani, F. Fortunato, E. Frattini, E. Abati, C. Bergamini, R. Fato, S. Tabano, M. Miozzo, G. Serratto, M. Passafaro, M. Deleidi, R. Silipigni, M. Nizzardo, N. Bresolin, G. Comi, S. Corti, C. Quinzii, A. Di Fonzo
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