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Review

Tackling Dysfunction of Mitochondrial Bioenergetics in the Brain

1
Department of Medical Basic Sciences, Neurosciences and Sense Organs, University of Bari Aldo Moro, 70124 Bari, Italy
2
IRCCS Fondazione Stella Maris, Calambrone, 56128 Pisa, Italy
*
Authors to whom correspondence should be addressed.
Academic Editors: Anna Atlante and Daniela Valenti
Int. J. Mol. Sci. 2021, 22(15), 8325; https://doi.org/10.3390/ijms22158325
Received: 16 June 2021 / Revised: 29 July 2021 / Accepted: 30 July 2021 / Published: 3 August 2021
Oxidative phosphorylation (OxPhos) is the basic function of mitochondria, although the landscape of mitochondrial functions is continuously growing to include more aspects of cellular homeostasis. Thanks to the application of -omics technologies to the study of the OxPhos system, novel features emerge from the cataloging of novel proteins as mitochondrial thus adding details to the mitochondrial proteome and defining novel metabolic cellular interrelations, especially in the human brain. We focussed on the diversity of bioenergetics demand and different aspects of mitochondrial structure, functions, and dysfunction in the brain. Definition such as ‘mitoexome’, ‘mitoproteome’ and ‘mitointeractome’ have entered the field of ‘mitochondrial medicine’. In this context, we reviewed several genetic defects that hamper the last step of aerobic metabolism, mostly involving the nervous tissue as one of the most prominent energy-dependent tissues and, as consequence, as a primary target of mitochondrial dysfunction. The dual genetic origin of the OxPhos complexes is one of the reasons for the complexity of the genotype-phenotype correlation when facing human diseases associated with mitochondrial defects. Such complexity clinically manifests with extremely heterogeneous symptoms, ranging from organ-specific to multisystemic dysfunction with different clinical courses. Finally, we briefly discuss the future directions of the multi-omics study of human brain disorders. View Full-Text
Keywords: mitochondria; mitochondrial DNA; nervous tissue; OxPhos complexes; bioenergetics; genomics; proteomics; mitochondrial diseases mitochondria; mitochondrial DNA; nervous tissue; OxPhos complexes; bioenergetics; genomics; proteomics; mitochondrial diseases
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MDPI and ACS Style

Zanfardino, P.; Doccini, S.; Santorelli, F.M.; Petruzzella, V. Tackling Dysfunction of Mitochondrial Bioenergetics in the Brain. Int. J. Mol. Sci. 2021, 22, 8325. https://doi.org/10.3390/ijms22158325

AMA Style

Zanfardino P, Doccini S, Santorelli FM, Petruzzella V. Tackling Dysfunction of Mitochondrial Bioenergetics in the Brain. International Journal of Molecular Sciences. 2021; 22(15):8325. https://doi.org/10.3390/ijms22158325

Chicago/Turabian Style

Zanfardino, Paola, Stefano Doccini, Filippo M. Santorelli, and Vittoria Petruzzella. 2021. "Tackling Dysfunction of Mitochondrial Bioenergetics in the Brain" International Journal of Molecular Sciences 22, no. 15: 8325. https://doi.org/10.3390/ijms22158325

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