Mitochondrial proteases: multifaceted regulators of mitochondrial plasticity

S Deshwal, KU Fiedler, T Langer - Annual review of …, 2020 - annualreviews.org
S Deshwal, KU Fiedler, T Langer
Annual review of biochemistry, 2020annualreviews.org
Mitochondria are essential metabolic hubs that dynamically adapt to physiological demands.
More than 40 proteases residing in different compartments of mitochondria, termed
mitoproteases, preserve mitochondrial proteostasis and are emerging as central regulators
of mitochondrial plasticity. These multifaceted enzymes limit the accumulation of short-lived,
regulatory proteins within mitochondria, modulate the activity of mitochondrial proteins by
protein processing, and mediate the degradation of damaged proteins. Various signaling …
Mitochondria are essential metabolic hubs that dynamically adapt to physiological demands. More than 40 proteases residing in different compartments of mitochondria, termed mitoproteases, preserve mitochondrial proteostasis and are emerging as central regulators of mitochondrial plasticity. These multifaceted enzymes limit the accumulation of short-lived, regulatory proteins within mitochondria, modulate the activity of mitochondrial proteins by protein processing, and mediate the degradation of damaged proteins. Various signaling cascades coordinate the activity of mitoproteases to preserve mitochondrial homeostasis and ensure cell survival. Loss of mitoproteases severely impairs the functional integrity of mitochondria, is associated with aging, and causes pleiotropic diseases. Understanding the dual function of mitoproteases as regulatory and quality control enzymes will help unravel the role of mitochondrial plasticity in aging and disease.
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