Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition.

Cell Death Dis. 2018 Dec 05;9(12):1168

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The mitochondrial Unfolded Protein Response (UPR^mt) pathway confers protection from misfolded and aggregated proteins by activating factors that promote protein folding and degradation. Our recent work on Lon protease, a member of the mitochondrial ATPase Associated with diverse cellular Activities (AAA⁺) family of mitochondrial resident proteases, suggests that mitochondrial translational inhibition may also be a feature of the UPR^mt pathway.

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Inactivation of Lon protease reveals a link between mitochondrial unfolded protein stress and mitochondrial translation inhibition.

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