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Mitochondrial UPR: A Double-Edged Sword.

Trends Cell Biol. 2016 Aug;26(8):563-5

Authors: Tian Y, Merkwirth C, Dillin A

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

PMID: 27394966 [PubMed – indexed for MEDLINE]