Mitochondrial damage control: delineating the mechanisms of mitochondrial repair

Mitochondrial function is essential for our health. The mitochondrial quality control machinery identifies and remedies dysfunctional mitochondria to protect the health of our mitochondrial network. The PINK1/Parkin pathway of the quality control machinery degrades severely damaged mitochondria, while the mitochondrial unfolded protein response (UPRmt) repairs mitochondria by increasing the production of mito-protective factors during proteostasis stress. While we have a detailed mechanistic understanding of PINK1/Parkin mitophagy, the UPRmt is not well understood in humans. The work in this thesis furthers our mechanistic understanding of the mammalian UPRmt and the signalling interplay between the UPRmt and PINK1/Parkin mitophagy.