Mitochondrial UPR: A Double-Edged Sword

The mitochondria, unfolded protein response (UPRmt) promotes the recovery of dysfunctional mitochondria. Surprisingly, UPRmt activation inadvertently maintains and propagates the deleterious mtDNA in a heteroplasmic Caenorhabditis elegans strain, with detrimental consequences. This study extends our understanding of the UPRmt and provides a possible therapeutic target for diseases associated with mtDNA mutations.