Unveiling the intercompartmental signaling axis: Mitochondrial to ER Stress Response (MERSR) and its impact on proteostasis.

Maintaining protein homeostasis is essential for cellular health. Our previous research uncovered a cross-compartmental Mitochondrial to Cytosolic Stress Response, activated by the perturbation of mitochondrial proteostasis, which ultimately results in the improvement of proteostasis in the cytosol....

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Main Authors: Jeson J Li, Nan Xin, Chunxia Yang, Bo G Kim, Larissa A Tavizon, Ruth Hong, Jina Park, Travis I Moore, Rebecca George Tharyan, Adam Antebi, Hyun-Eui Kim
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2025-05-01
Series:PLoS Genetics
Online Access:https://doi.org/10.1371/journal.pgen.1011700
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author Jeson J Li
Nan Xin
Chunxia Yang
Bo G Kim
Larissa A Tavizon
Ruth Hong
Jina Park
Travis I Moore
Rebecca George Tharyan
Adam Antebi
Hyun-Eui Kim
author_facet Jeson J Li
Nan Xin
Chunxia Yang
Bo G Kim
Larissa A Tavizon
Ruth Hong
Jina Park
Travis I Moore
Rebecca George Tharyan
Adam Antebi
Hyun-Eui Kim
author_sort Jeson J Li
collection DOAJ
description Maintaining protein homeostasis is essential for cellular health. Our previous research uncovered a cross-compartmental Mitochondrial to Cytosolic Stress Response, activated by the perturbation of mitochondrial proteostasis, which ultimately results in the improvement of proteostasis in the cytosol. Here, we found that this signaling axis also influences the unfolded protein response of the endoplasmic reticulum (UPRER), suggesting the presence of a Mitochondria to ER Stress Response (MERSR). During MERSR, the IRE1 branch of UPRER is inhibited, introducing a previously unknown regulatory component of MCSR. Moreover, proteostasis is enhanced through the upregulation of the PERK-eIF2α signaling pathway, increasing phosphorylation of eIF2α and improving the ER's ability to handle proteostasis. MERSR activation in both polyglutamine and amyloid-beta peptide-expressing C. elegans disease models also led to improvement in both aggregate burden and overall disease outcome. These findings shed light on the coordination between the mitochondria and the ER in maintaining cellular proteostasis and provide further evidence for the importance of intercompartmental signaling.
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publisher Public Library of Science (PLoS)
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spelling doaj-art-e02446e1edcc4b0eb04cb0e8a3fa44202025-08-20T01:52:54ZengPublic Library of Science (PLoS)PLoS Genetics1553-73901553-74042025-05-01215e101170010.1371/journal.pgen.1011700Unveiling the intercompartmental signaling axis: Mitochondrial to ER Stress Response (MERSR) and its impact on proteostasis.Jeson J LiNan XinChunxia YangBo G KimLarissa A TavizonRuth HongJina ParkTravis I MooreRebecca George TharyanAdam AntebiHyun-Eui KimMaintaining protein homeostasis is essential for cellular health. Our previous research uncovered a cross-compartmental Mitochondrial to Cytosolic Stress Response, activated by the perturbation of mitochondrial proteostasis, which ultimately results in the improvement of proteostasis in the cytosol. Here, we found that this signaling axis also influences the unfolded protein response of the endoplasmic reticulum (UPRER), suggesting the presence of a Mitochondria to ER Stress Response (MERSR). During MERSR, the IRE1 branch of UPRER is inhibited, introducing a previously unknown regulatory component of MCSR. Moreover, proteostasis is enhanced through the upregulation of the PERK-eIF2α signaling pathway, increasing phosphorylation of eIF2α and improving the ER's ability to handle proteostasis. MERSR activation in both polyglutamine and amyloid-beta peptide-expressing C. elegans disease models also led to improvement in both aggregate burden and overall disease outcome. These findings shed light on the coordination between the mitochondria and the ER in maintaining cellular proteostasis and provide further evidence for the importance of intercompartmental signaling.https://doi.org/10.1371/journal.pgen.1011700
spellingShingle Jeson J Li
Nan Xin
Chunxia Yang
Bo G Kim
Larissa A Tavizon
Ruth Hong
Jina Park
Travis I Moore
Rebecca George Tharyan
Adam Antebi
Hyun-Eui Kim
Unveiling the intercompartmental signaling axis: Mitochondrial to ER Stress Response (MERSR) and its impact on proteostasis.
PLoS Genetics
title Unveiling the intercompartmental signaling axis: Mitochondrial to ER Stress Response (MERSR) and its impact on proteostasis.
title_full Unveiling the intercompartmental signaling axis: Mitochondrial to ER Stress Response (MERSR) and its impact on proteostasis.
title_fullStr Unveiling the intercompartmental signaling axis: Mitochondrial to ER Stress Response (MERSR) and its impact on proteostasis.
title_full_unstemmed Unveiling the intercompartmental signaling axis: Mitochondrial to ER Stress Response (MERSR) and its impact on proteostasis.
title_short Unveiling the intercompartmental signaling axis: Mitochondrial to ER Stress Response (MERSR) and its impact on proteostasis.
title_sort unveiling the intercompartmental signaling axis mitochondrial to er stress response mersr and its impact on proteostasis
url https://doi.org/10.1371/journal.pgen.1011700
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