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

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.