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Mitochondrial Structure and Bioenergetics in Normal and Disease Conditions

1
Mitochondrial Biology Unit, The MRC and University of Cambridge, Cambridge CB2 0XY, UK
2
Department of Neurosciences, University of Padova, 35128 Padova, Italy
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2021, 22(2), 586; https://doi.org/10.3390/ijms22020586
Received: 16 December 2020 / Revised: 3 January 2021 / Accepted: 4 January 2021 / Published: 8 January 2021
Mitochondria are ubiquitous intracellular organelles found in almost all eukaryotes and involved in various aspects of cellular life, with a primary role in energy production. The interest in this organelle has grown stronger with the discovery of their link to various pathologies, including cancer, aging and neurodegenerative diseases. Indeed, dysfunctional mitochondria cannot provide the required energy to tissues with a high-energy demand, such as heart, brain and muscles, leading to a large spectrum of clinical phenotypes. Mitochondrial defects are at the origin of a group of clinically heterogeneous pathologies, called mitochondrial diseases, with an incidence of 1 in 5000 live births. Primary mitochondrial diseases are associated with genetic mutations both in nuclear and mitochondrial DNA (mtDNA), affecting genes involved in every aspect of the organelle function. As a consequence, it is difficult to find a common cause for mitochondrial diseases and, subsequently, to offer a precise clinical definition of the pathology. Moreover, the complexity of this condition makes it challenging to identify possible therapies or drug targets. View Full-Text
Keywords: ATP production; biogenesis of the respiratory chain; mitochondrial disease; mi-tochondrial electrochemical gradient; mitochondrial potential; mitochondrial proton pumping; mitochondrial respiratory chain; oxidative phosphorylation; respiratory complex; respiratory supercomplex ATP production; biogenesis of the respiratory chain; mitochondrial disease; mi-tochondrial electrochemical gradient; mitochondrial potential; mitochondrial proton pumping; mitochondrial respiratory chain; oxidative phosphorylation; respiratory complex; respiratory supercomplex
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MDPI and ACS Style

Protasoni, M.; Zeviani, M. Mitochondrial Structure and Bioenergetics in Normal and Disease Conditions. Int. J. Mol. Sci. 2021, 22, 586. https://doi.org/10.3390/ijms22020586

AMA Style

Protasoni M, Zeviani M. Mitochondrial Structure and Bioenergetics in Normal and Disease Conditions. International Journal of Molecular Sciences. 2021; 22(2):586. https://doi.org/10.3390/ijms22020586

Chicago/Turabian Style

Protasoni, Margherita; Zeviani, Massimo. 2021. "Mitochondrial Structure and Bioenergetics in Normal and Disease Conditions" Int. J. Mol. Sci. 22, no. 2: 586. https://doi.org/10.3390/ijms22020586

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