Journal
CELLS
Volume 7, Issue 10, Pages -Publisher
MDPI
DOI: 10.3390/cells7100163
Keywords
AAA protease; ATP-dependent proteolysis; mitochondria; inner mitochondrial membrane proteostasis; m-AAA protease; i-AAA protease; neurodegenerative diseases
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Funding
- National Science Centre (NCN) [2015/16/S/NZ3/00364]
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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.
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