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Review

Interplay between Mitochondrial Protein Import and Respiratory Complexes Assembly in Neuronal Health and Degeneration

1
School of Biochemistry, University of Bristol, Bristol BS8 1TD, UK
2
Medical Research Council-Mitochondrial Biology Unit, University of Cambridge, Cambridge CB2 0XY, UK
3
Centre for Neuroscience and Regenerative Medicine, Faculty of Science, University of Technology Sydney, Ultimo, NSW 2007, Australia
*
Authors to whom correspondence should be addressed.
Academic Editors: Giorgio Lenaz and Salvatore Nesci
Life 2021, 11(5), 432; https://doi.org/10.3390/life11050432
Received: 25 March 2021 / Revised: 27 April 2021 / Accepted: 2 May 2021 / Published: 11 May 2021
(This article belongs to the Special Issue Impaired Mitochondrial Bioenergetics under Pathological Conditions)
The fact that >99% of mitochondrial proteins are encoded by the nuclear genome and synthesised in the cytosol renders the process of mitochondrial protein import fundamental for normal organelle physiology. In addition to this, the nuclear genome comprises most of the proteins required for respiratory complex assembly and function. This means that without fully functional protein import, mitochondrial respiration will be defective, and the major cellular ATP source depleted. When mitochondrial protein import is impaired, a number of stress response pathways are activated in order to overcome the dysfunction and restore mitochondrial and cellular proteostasis. However, prolonged impaired mitochondrial protein import and subsequent defective respiratory chain function contributes to a number of diseases including primary mitochondrial diseases and neurodegeneration. This review focuses on how the processes of mitochondrial protein translocation and respiratory complex assembly and function are interlinked, how they are regulated, and their importance in health and disease. View Full-Text
Keywords: protein import; mitochondrial dysfunction; respiratory complex assembly; supercomplexes; neurodegeneration; mitochondrial proteostasis protein import; mitochondrial dysfunction; respiratory complex assembly; supercomplexes; neurodegeneration; mitochondrial proteostasis
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MDPI and ACS Style

Needs, H.I.; Protasoni, M.; Henley, J.M.; Prudent, J.; Collinson, I.; Pereira, G.C. Interplay between Mitochondrial Protein Import and Respiratory Complexes Assembly in Neuronal Health and Degeneration. Life 2021, 11, 432. https://doi.org/10.3390/life11050432

AMA Style

Needs HI, Protasoni M, Henley JM, Prudent J, Collinson I, Pereira GC. Interplay between Mitochondrial Protein Import and Respiratory Complexes Assembly in Neuronal Health and Degeneration. Life. 2021; 11(5):432. https://doi.org/10.3390/life11050432

Chicago/Turabian Style

Needs, Hope I., Margherita Protasoni, Jeremy M. Henley, Julien Prudent, Ian Collinson, and Gonçalo C. Pereira. 2021. "Interplay between Mitochondrial Protein Import and Respiratory Complexes Assembly in Neuronal Health and Degeneration" Life 11, no. 5: 432. https://doi.org/10.3390/life11050432

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