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The Mitochondrial Lon Protease: Novel Functions off the Beaten Track?

Institute for Biochemistry and Molecular Biology (IBMB), University of Bonn, Nussallee 11, D-53115 Bonn, Germany
Author to whom correspondence should be addressed.
Biomolecules 2020, 10(2), 253;
Received: 16 December 2019 / Revised: 3 February 2020 / Accepted: 4 February 2020 / Published: 7 February 2020
To maintain organellar function, mitochondria contain an elaborate endogenous protein quality control system. As one of the two soluble energy-dependent proteolytic enzymes in the matrix compartment, the protease Lon is a major component of this system, responsible for the degradation of misfolded proteins, in particular under oxidative stress conditions. Lon defects have been shown to negatively affect energy production by oxidative phosphorylation but also mitochondrial gene expression. In this review, recent studies on the role of Lon in mammalian cells, in particular on its protective action under diverse stress conditions and its relationship to important human diseases are summarized and commented.
Keywords: mitochondria; Lon protease; protein quality control mitochondria; Lon protease; protein quality control
MDPI and ACS Style

Voos, W.; Pollecker, K. The Mitochondrial Lon Protease: Novel Functions off the Beaten Track? Biomolecules 2020, 10, 253.

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