线粒体 UPR：一把双刃剑。

Mitochondrial UPR: A Double-Edged Sword.

机构信息

Howard Hughes Medical Institute and Department of Molecular and Cell Biology, Glenn Center for Aging Research, University of California, Berkeley, Berkeley, CA 94720, USA.

出版信息

Trends Cell Biol. 2016 Aug;26(8):563-565. doi: 10.1016/j.tcb.2016.06.006. Epub 2016 Jul 6.

DOI:10.1016/j.tcb.2016.06.006

PMID:27394966

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.

摘要

线粒体未折叠蛋白反应（UPR(mt)）可促进功能失调的线粒体的恢复。令人惊讶的是，UPR(mt) 的激活无意中维持和传播了异质 C. elegans 菌株中有害的 mtDNA，带来了有害的后果。这项研究扩展了我们对 UPR(mt) 的理解，并为与 mtDNA 突变相关的疾病提供了一个可能的治疗靶点。