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Regulated Proteolysis in Microorganisms pp 223–263Cite as

The Role of AAA+ Proteases in Mitochondrial Protein Biogenesis, Homeostasis and Activity Control

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Part of the book series: Subcellular Biochemistry ((SCBI,volume 66))

Abstract

Mitochondria are specialised organelles that are structurally and functionally integrated into cells in the vast majority of eukaryotes. They are the site of numerous enzymatic reactions, some of which are essential for life. The double lipid membrane of the mitochondrion, that spatially defines the organelle and is necessary for some functions, also creates a physical but semi-permeable barrier to the rest of the cell. Thus to ensure the biogenesis, regulation and maintenance of a functional population of proteins, an autonomous protein handling network within mitochondria is required. This includes resident mitochondrial protein translocation machinery, processing peptidases, molecular chaperones and proteases. This review highlights the contribution of proteases of the AAA+ superfamily to protein quality and activity control within the mitochondrion. Here they are responsible for the degradation of unfolded, unassembled and oxidatively damaged proteins as well as the activity control of some enzymes. Since most knowledge about these proteases has been gained from studies in the eukaryotic microorganism Saccharomyces cerevisiae, much of the discussion here centres on their role in this organism. However, reference is made to mitochondrial AAA+ proteases in other organisms, particularly in cases where they play a unique role such as the mitochondrial unfolded protein response. As these proteases influence mitochondrial function in both health and disease in humans, an understanding of their regulation and diverse activities is necessary.

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Authors and Affiliations

  1. Institut für Biochemie und Molekularbiologie (IBMB), Universität Bonn, Nussallee 11, 53115, Bonn, Germany

    Wolfgang Voos

  2. Department of Biochemistry, La Trobe Institute for Molecular Science (LIMS), La Trobe University, Melbourne, VIC, 3086, Australia

    Linda A. Ward & Kaye N. Truscott

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  1. Wolfgang Voos
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  3. Kaye N. Truscott
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Correspondence to Wolfgang Voos or Kaye N. Truscott .

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    David A. Dougan

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Voos, W., Ward, L.A., Truscott, K.N. (2013). The Role of AAA+ Proteases in Mitochondrial Protein Biogenesis, Homeostasis and Activity Control. In: Dougan, D. (eds) Regulated Proteolysis in Microorganisms. Subcellular Biochemistry, vol 66. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-5940-4_9

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