Neuronal disorders are associated with a profound loss of mitochondrial functions caused by various stress conditions,such as oxidative and metabolic stress,protein folding or import defects,and mitochondrial DNA alte...Neuronal disorders are associated with a profound loss of mitochondrial functions caused by various stress conditions,such as oxidative and metabolic stress,protein folding or import defects,and mitochondrial DNA alteration.Cells engage in different coordinated responses to safeguard mitochondrial homeostasis.In this review,we will explore the contribution of mitochondrial stress responses that are activated by the organelle to perceive these dangerous conditions,keep them under control and rescue the physiological condition of nervous cells.In the sections to come,particular attention will be dedicated to analyzing how compensatory mitochondrial hyperfusion,mitophagy,mitochondrial unfolding protein response,and apoptosis impact human neuronal diseases.Finally,we will discuss the relevance of the new concept:the“mito-inflammation”,a mitochondria-mediated inflammatory response that is recently found to cover a relevant role in the pathogenesis of diverse inflammatory-related diseases,including neuronal disorders.展开更多
基金supported by grants from local funds from University of Ferrara, FIR-2021the Italian Ministry of Health(GR-2016-02364602)+4 种基金the Italian Ministry of Education, University and Research(PRIN Grant 2017XA5 J5 N)(to AR)grants from Italian Association for Cancer Research(AIRC, IG-23670)Telethon(GGP11139 B)local funds from the University of Ferrarathe Italian Ministry of Education, University and Research(PRIN Grant 2017 E5 L5 P3)(to PP): Associazione Ricerca Oncologica Sperimentale Estense(AROSE)
文摘Neuronal disorders are associated with a profound loss of mitochondrial functions caused by various stress conditions,such as oxidative and metabolic stress,protein folding or import defects,and mitochondrial DNA alteration.Cells engage in different coordinated responses to safeguard mitochondrial homeostasis.In this review,we will explore the contribution of mitochondrial stress responses that are activated by the organelle to perceive these dangerous conditions,keep them under control and rescue the physiological condition of nervous cells.In the sections to come,particular attention will be dedicated to analyzing how compensatory mitochondrial hyperfusion,mitophagy,mitochondrial unfolding protein response,and apoptosis impact human neuronal diseases.Finally,we will discuss the relevance of the new concept:the“mito-inflammation”,a mitochondria-mediated inflammatory response that is recently found to cover a relevant role in the pathogenesis of diverse inflammatory-related diseases,including neuronal disorders.
