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Quality control of the mitochondrion

Matthew Ng 1 Timothy Wai 2, * Anne Simonsen 1, *
* Corresponding author
2 Biologie mitochondriale – Mitochondrial biology
CNRS - Centre National de la Recherche Scientifique : UMR3691, UP - Université de Paris, Institut Pasteur [Paris]
Abstract : Mitochondria are essential organelles that execute and coordinate various metabolic processes in the cell. Mitochondrial dysfunction severely affects cell fitness and contributes to disease. Proper organellar function depends on the biogenesis and maintenance of mitochondria and its >1,000 proteins. As a result, the cell has evolved mechanisms to coordinate protein and organellar quality control, such as the turnover of proteins via mitochondria-associated degradation, the ubiquitin-proteasome system, and mitoproteases, as well as the elimination of mitochondria through mitophagy. Specific quality control mechanisms are engaged depending upon the nature and severity of mitochondrial dysfunction, which can also feed back to elicit transcriptional or proteomic remodeling by the cell. Here, we will discuss the current understanding of how these different quality control mechanisms are integrated and overlap to maintain protein and organellar quality and how they may be relevant for cellular and organismal health.
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Submitted on : Monday, June 28, 2021 - 3:05:17 PM
Last modification on : Tuesday, July 13, 2021 - 3:15:08 AM

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Matthew Ng, Timothy Wai, Anne Simonsen. Quality control of the mitochondrion. Developmental Cell, Elsevier, 2021, 56 (7), pp.881 - 905. ⟨10.1016/j.devcel.2021.02.009⟩. ⟨pasteur-03272624⟩

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