Open AccessThe “mitochondrial stress responses”: the “Dr. Jekyll and Mr. Hyde” of neuronal disorders
Author(s) -
Simone Patergnani,
Giampaolo Morciano,
Marianna Carinci,
Sara Leo,
Paolo Pinton,
Alessandro Rimessi
Publication year - 2022
Publication title -
neural regeneration research/neural regeneration research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.93
H-Index - 38
eISSN - 1876-7958
pISSN - 1673-5374
DOI - 10.4103/1673-5374.339473
Subject(s) - mitophagy , mitochondrion , neuroscience , oxidative stress , microbiology and biotechnology , biology , mitochondrial dna , mitochondrial fusion , cellular stress response , inflammation , neurodegeneration , dnaja3 , unfolded protein response , apoptosis , fight or flight response , medicine , immunology , autophagy , genetics , biochemistry , pathology , endoplasmic reticulum , gene , disease
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.