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AAA Proteases: Guardians of Mitochondrial Function and Homeostasis

Department of Cellular Molecular Biology, Faculty of Biotechnology, University of Wroclaw, F. Joliot-Curie 14A, 50-383 Wroclaw, Poland
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Cells 2018, 7(10), 163; https://doi.org/10.3390/cells7100163
Received: 18 September 2018 / Revised: 4 October 2018 / Accepted: 9 October 2018 / Published: 11 October 2018
(This article belongs to the Special Issue Mitochondrial Biology in Health and Disease)
Mitochondria are dynamic, semi-autonomous organelles that execute numerous life-sustaining tasks in eukaryotic cells. Functioning of mitochondria depends on the adequate action of versatile proteinaceous machineries. Fine-tuning of mitochondrial activity in response to cellular needs involves continuous remodeling of organellar proteome. This process not only includes modulation of various biogenetic pathways, but also the removal of superfluous proteins by adenosine triphosphate (ATP)-driven proteolytic machineries. Accordingly, all mitochondrial sub-compartments are under persistent surveillance of ATP-dependent proteases. Particularly important are highly conserved two inner mitochondrial membrane-bound metalloproteases known as m-AAA and i-AAA (ATPases associated with diverse cellular activities), whose mis-functioning may lead to impaired organellar function and consequently to development of severe diseases. Herein, we discuss the current knowledge of yeast, mammalian, and plant AAA proteases and their implications in mitochondrial function and homeostasis maintenance. View Full-Text
Keywords: AAA protease; ATP-dependent proteolysis; mitochondria; inner mitochondrial membrane proteostasis; m-AAA protease; i-AAA protease; neurodegenerative diseases AAA protease; ATP-dependent proteolysis; mitochondria; inner mitochondrial membrane proteostasis; m-AAA protease; i-AAA protease; neurodegenerative diseases
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Opalińska, M.; Jańska, H. AAA Proteases: Guardians of Mitochondrial Function and Homeostasis. Cells 2018, 7, 163.

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