Institute for Research in Biomedicine (IRB Barcelona), Barcelona, Spain.
EMBO J. 2013 Aug 28;32(17):2348-61. doi: 10.1038/emboj.2013.168. Epub 2013 Aug 6.
Mitofusin 2 (Mfn2) is a key protein in mitochondrial fusion and it participates in the bridging of mitochondria to the endoplasmic reticulum (ER). Recent data indicate that Mfn2 ablation leads to ER stress. Here we report on the mechanisms by which Mfn2 modulates cellular responses to ER stress. Induction of ER stress in Mfn2-deficient cells caused massive ER expansion and excessive activation of all three Unfolded Protein Response (UPR) branches (PERK, XBP-1, and ATF6). In spite of an enhanced UPR, these cells showed reduced activation of apoptosis and autophagy during ER stress. Silencing of PERK increased the apoptosis of Mfn2-ablated cells in response to ER stress. XBP-1 loss-of-function ameliorated autophagic activity of these cells upon ER stress. Mfn2 physically interacts with PERK, and Mfn2-ablated cells showed sustained activation of this protein kinase under basal conditions. Unexpectedly, PERK silencing in these cells reduced ROS production, normalized mitochondrial calcium, and improved mitochondrial morphology. In summary, our data indicate that Mfn2 is an upstream modulator of PERK. Furthermore, Mfn2 loss-of-function reveals that PERK is a key regulator of mitochondrial morphology and function.
线粒体融合蛋白 2(Mfn2)是线粒体融合的关键蛋白,它参与线粒体与内质网(ER)的连接。最近的数据表明,Mfn2 的缺失会导致内质网应激。在这里,我们报告了 Mfn2 调节细胞对内质网应激反应的机制。在 Mfn2 缺陷细胞中诱导内质网应激会导致内质网大量扩张和所有三种未折叠蛋白反应(UPR)分支(PERK、XBP-1 和 ATF6)的过度激活。尽管 UPR 增强,这些细胞在 ER 应激期间表现出凋亡和自噬的激活减少。PERK 的沉默增加了 Mfn2 缺失细胞对 ER 应激的凋亡。XBP-1 功能丧失可改善这些细胞在 ER 应激时的自噬活性。Mfn2 与 PERK 发生物理相互作用,并且 Mfn2 缺失细胞在基础条件下持续激活这种蛋白激酶。出乎意料的是,这些细胞中 PERK 的沉默减少了 ROS 的产生,使线粒体钙正常化,并改善了线粒体形态。总之,我们的数据表明 Mfn2 是 PERK 的上游调节剂。此外,Mfn2 缺失功能表明 PERK 是线粒体形态和功能的关键调节剂。