The “mitochondrial stress responses”: the “Dr. Jekyll and Mr. Hyde” of neuronal disorders
Open Access
- 1 January 2022
- journal article
- review article
- Published by Medknow in Neural Regeneration Research
- Vol. 17 (12), 2563-2575
- https://doi.org/10.4103/1673-5374.339473
Abstract
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.Keywords
This publication has 306 references indexed in Scilit:
- Mitonuclear protein imbalance as a conserved longevity mechanismNature, 2013
- Mitochondrial Ca2+ and apoptosisCell Calcium, 2012
- Genetic analysis of mitochondrial protein misfolding in Drosophila melanogasterCell Death & Differentiation, 2012
- Mitochondria in NeurodegenerationAdvances in experimental medicine and biology, 2011
- NLRX1 Negatively Regulates TLR-Induced NF-κB Signaling by Targeting TRAF6 and IKKImmunity, 2011
- Mitochondrial Quality Control and Parkinson’s Disease: A Pathway UnfoldsMolecular Neurobiology, 2010
- Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALSNeuron, 2010
- Nix is a selective autophagy receptor for mitochondrial clearanceEMBO Reports, 2009
- SLP-2 is required for stress-induced mitochondrial hyperfusionThe EMBO Journal, 2009
- Mitochondrial dysfunction and oxidative stress in neurodegenerative diseasesNature, 2006