Suppr超能文献

Mfn2 通过抑制 PERK 调节 UPR 和线粒体功能。

Mfn2 modulates the UPR and mitochondrial function via repression of PERK.

机构信息

Institute for Research in Biomedicine (IRB Barcelona), Barcelona, Spain.

出版信息

EMBO J. 2013 Aug 28;32(17):2348-61. doi: 10.1038/emboj.2013.168. Epub 2013 Aug 6.

Abstract

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.

摘要

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。

相似文献

1
Mfn2 modulates the UPR and mitochondrial function via repression of PERK.
EMBO J. 2013 Aug 28;32(17):2348-61. doi: 10.1038/emboj.2013.168. Epub 2013 Aug 6.
5
UPR-induced resistance to etoposide is downstream of PERK and independent of changes in topoisomerase IIα levels.
PLoS One. 2012;7(10):e47931. doi: 10.1371/journal.pone.0047931. Epub 2012 Oct 29.
6
PI 3-kinase regulatory subunits as regulators of the unfolded protein response.
Methods Enzymol. 2011;490:147-58. doi: 10.1016/B978-0-12-385114-7.00009-X.
9
The eIF2 kinase PERK and the integrated stress response facilitate activation of ATF6 during endoplasmic reticulum stress.
Mol Biol Cell. 2011 Nov;22(22):4390-405. doi: 10.1091/mbc.E11-06-0510. Epub 2011 Sep 14.
10
The unfolded protein response in melanocytes: activation in response to chemical stressors of the endoplasmic reticulum and tyrosinase misfolding.
Pigment Cell Melanoma Res. 2010 Oct;23(5):627-34. doi: 10.1111/j.1755-148X.2010.00718.x. Epub 2010 Apr 23.

引用本文的文献

2
The role of mitochondria-associated ER membranes in disease pathology: protein complex and therapeutic targets.
Front Cell Dev Biol. 2025 Jun 30;13:1629568. doi: 10.3389/fcell.2025.1629568. eCollection 2025.
5
Mitochondria-Associated Endoplasmic Reticulum Membranes in Human Health and Diseases.
MedComm (2020). 2025 Jun 27;6(7):e70259. doi: 10.1002/mco2.70259. eCollection 2025 Jul.
7
Correlation of organelle interactions in the development of non-alcoholic fatty liver disease.
Front Immunol. 2025 Apr 16;16:1567743. doi: 10.3389/fimmu.2025.1567743. eCollection 2025.
9
MAM kinases: physiological roles, related diseases, and therapeutic perspectives-a systematic review.
Cell Mol Biol Lett. 2025 Mar 28;30(1):35. doi: 10.1186/s11658-025-00714-w.

本文引用的文献

1
Stress-induced phosphorylation and proteasomal degradation of mitofusin 2 facilitates mitochondrial fragmentation and apoptosis.
Mol Cell. 2012 Aug 24;47(4):547-57. doi: 10.1016/j.molcel.2012.05.041. Epub 2012 Jun 28.
2
PERK is required at the ER-mitochondrial contact sites to convey apoptosis after ROS-based ER stress.
Cell Death Differ. 2012 Nov;19(11):1880-91. doi: 10.1038/cdd.2012.74. Epub 2012 Jun 15.
3
Central role of mitofusin 2 in autophagosome-lysosome fusion in cardiomyocytes.
J Biol Chem. 2012 Jul 6;287(28):23615-25. doi: 10.1074/jbc.M112.379164. Epub 2012 May 22.
4
Loss of mitofusin 2 promotes endoplasmic reticulum stress.
J Biol Chem. 2012 Jun 8;287(24):20321-32. doi: 10.1074/jbc.M112.359174. Epub 2012 Apr 17.
5
Bax regulates primary necrosis through mitochondrial dynamics.
Proc Natl Acad Sci U S A. 2012 Apr 24;109(17):6566-71. doi: 10.1073/pnas.1201608109. Epub 2012 Apr 9.
6
Mitofusin 2 (Mfn2) links mitochondrial and endoplasmic reticulum function with insulin signaling and is essential for normal glucose homeostasis.
Proc Natl Acad Sci U S A. 2012 Apr 3;109(14):5523-8. doi: 10.1073/pnas.1108220109. Epub 2012 Mar 16.
7
Inhibition of mitochondrial fission prevents cell cycle progression in lung cancer.
FASEB J. 2012 May;26(5):2175-86. doi: 10.1096/fj.11-196543. Epub 2012 Feb 9.
8
The eIF2 kinase PERK and the integrated stress response facilitate activation of ATF6 during endoplasmic reticulum stress.
Mol Biol Cell. 2011 Nov;22(22):4390-405. doi: 10.1091/mbc.E11-06-0510. Epub 2011 Sep 14.
9
ER tubules mark sites of mitochondrial division.
Science. 2011 Oct 21;334(6054):358-62. doi: 10.1126/science.1207385. Epub 2011 Sep 1.
10
The role of Nogo and the mitochondria-endoplasmic reticulum unit in pulmonary hypertension.
Sci Transl Med. 2011 Jun 22;3(88):88ra55. doi: 10.1126/scitranslmed.3002194.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验