Inhibition of Rho GEFs attenuates pulmonary fibrosis through suppressing myofibroblast activation and reprogramming profibrotic macrophages

Abstract Idiopathic pulmonary fibrosis has a poor prognosis, with existing medications only partially alleviating symptoms, highlighting the urgent need for new therapeutic approaches. The dysregulations of Rho GTPases/ROCK are related with various diseases, including fibrosis. Nevertheless, the dev...

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Main Authors: Chengju Luo, Chenqi Huang, Yuqi Zhu, Yuxin Zhou, Yansheng Qiao, Chenxiao Shi, Yuan Gao, Yongjian Guo, Libin Wei
Format: Article
Language:English
Published: Nature Publishing Group 2025-04-01
Series:Cell Death and Disease
Online Access:https://doi.org/10.1038/s41419-025-07573-5
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author Chengju Luo
Chenqi Huang
Yuqi Zhu
Yuxin Zhou
Yansheng Qiao
Chenxiao Shi
Yuan Gao
Yongjian Guo
Libin Wei
author_facet Chengju Luo
Chenqi Huang
Yuqi Zhu
Yuxin Zhou
Yansheng Qiao
Chenxiao Shi
Yuan Gao
Yongjian Guo
Libin Wei
author_sort Chengju Luo
collection DOAJ
description Abstract Idiopathic pulmonary fibrosis has a poor prognosis, with existing medications only partially alleviating symptoms, highlighting the urgent need for new therapeutic approaches. The dysregulations of Rho GTPases/ROCK are related with various diseases, including fibrosis. Nevertheless, the development of drugs for pulmonary fibrosis treatment has predominantly concentrated on ROCK inhibitors. Small GTPases have been historically recognized as “undruggable”. Here, we explore a novel Rho GEFs inhibitor GL-V9, and find that GL-V9 alleviates bleomycin-induced pulmonary fibrosis in mice by inhibiting myofibroblast activation and reprogramming profibrotic macrophages. Distinct from the mechanisms of the first-line drug Nintedanib, GL-V9 binds to the DH/PH domain of Rho GEFs and block the activation of Rho GTPase signaling. This action subsequently suppresses myofibroblast activation by interfering with Rho GTPase-dependent cytoskeletal reorganization and the activity of MRTF and YAP, and inhibits M2 macrophage polarization by modulating RhoA/STAT3 activity. The discovery of new regulatory mechanisms of GL-V9 suggests that targeting Rho GEFs represents a potent strategy for pulmonary fibrosis treatment.
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institution DOAJ
issn 2041-4889
language English
publishDate 2025-04-01
publisher Nature Publishing Group
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series Cell Death and Disease
spelling doaj-art-3abc82144b74405fbd23c00a024a2bfc2025-08-20T03:10:08ZengNature Publishing GroupCell Death and Disease2041-48892025-04-0116111310.1038/s41419-025-07573-5Inhibition of Rho GEFs attenuates pulmonary fibrosis through suppressing myofibroblast activation and reprogramming profibrotic macrophagesChengju Luo0Chenqi Huang1Yuqi Zhu2Yuxin Zhou3Yansheng Qiao4Chenxiao Shi5Yuan Gao6Yongjian Guo7Libin Wei8Jiangsu Key Laboratory of Carcinogenesis and Intervention, China Pharmaceutical UniversityJiangsu Key Laboratory of Carcinogenesis and Intervention, China Pharmaceutical UniversityBayi Hospital Affiliated to Nanjing University of Chinese MedicineJiangsu Key Laboratory of Carcinogenesis and Intervention, China Pharmaceutical UniversityJiangsu Key Laboratory of Carcinogenesis and Intervention, China Pharmaceutical UniversityJiangsu Key Laboratory of Carcinogenesis and Intervention, China Pharmaceutical UniversityPublic Laboratory Platform, China Pharmaceutical UniversitySchool of Biopharmacy, China Pharmaceutical UniversityJiangsu Key Laboratory of Carcinogenesis and Intervention, China Pharmaceutical UniversityAbstract Idiopathic pulmonary fibrosis has a poor prognosis, with existing medications only partially alleviating symptoms, highlighting the urgent need for new therapeutic approaches. The dysregulations of Rho GTPases/ROCK are related with various diseases, including fibrosis. Nevertheless, the development of drugs for pulmonary fibrosis treatment has predominantly concentrated on ROCK inhibitors. Small GTPases have been historically recognized as “undruggable”. Here, we explore a novel Rho GEFs inhibitor GL-V9, and find that GL-V9 alleviates bleomycin-induced pulmonary fibrosis in mice by inhibiting myofibroblast activation and reprogramming profibrotic macrophages. Distinct from the mechanisms of the first-line drug Nintedanib, GL-V9 binds to the DH/PH domain of Rho GEFs and block the activation of Rho GTPase signaling. This action subsequently suppresses myofibroblast activation by interfering with Rho GTPase-dependent cytoskeletal reorganization and the activity of MRTF and YAP, and inhibits M2 macrophage polarization by modulating RhoA/STAT3 activity. The discovery of new regulatory mechanisms of GL-V9 suggests that targeting Rho GEFs represents a potent strategy for pulmonary fibrosis treatment.https://doi.org/10.1038/s41419-025-07573-5
spellingShingle Chengju Luo
Chenqi Huang
Yuqi Zhu
Yuxin Zhou
Yansheng Qiao
Chenxiao Shi
Yuan Gao
Yongjian Guo
Libin Wei
Inhibition of Rho GEFs attenuates pulmonary fibrosis through suppressing myofibroblast activation and reprogramming profibrotic macrophages
Cell Death and Disease
title Inhibition of Rho GEFs attenuates pulmonary fibrosis through suppressing myofibroblast activation and reprogramming profibrotic macrophages
title_full Inhibition of Rho GEFs attenuates pulmonary fibrosis through suppressing myofibroblast activation and reprogramming profibrotic macrophages
title_fullStr Inhibition of Rho GEFs attenuates pulmonary fibrosis through suppressing myofibroblast activation and reprogramming profibrotic macrophages
title_full_unstemmed Inhibition of Rho GEFs attenuates pulmonary fibrosis through suppressing myofibroblast activation and reprogramming profibrotic macrophages
title_short Inhibition of Rho GEFs attenuates pulmonary fibrosis through suppressing myofibroblast activation and reprogramming profibrotic macrophages
title_sort inhibition of rho gefs attenuates pulmonary fibrosis through suppressing myofibroblast activation and reprogramming profibrotic macrophages
url https://doi.org/10.1038/s41419-025-07573-5
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