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Mitochondrial morphology and MAVS‐IFN1 signaling pathway in muscles of anti‐MDA5 dermatomyositis

OBJECTIVE: This study aimed to investigate mitochondrial changes and the mitochondrial antiviral‐signaling protein (MAVS)‐type I interferon (IFN1) signaling pathway in the muscles of anti‐melanoma differentiation gene 5(MDA5) dermatomyositis (DM) patients. METHODS: Eleven anti‐MDA5 DM and ten antibo...

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Autores principales: Jiang, Yanyan, Liu, Yilin, Zhao, Yawen, Zheng, Yiming, Yu, Meng, Deng, Jianwen, Hao, Hongjun, Zhang, Wei, Wang, Zhaoxia, Yuan, Yun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7951095/
https://www.ncbi.nlm.nih.gov/pubmed/33576578
http://dx.doi.org/10.1002/acn3.51311
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author Jiang, Yanyan
Liu, Yilin
Zhao, Yawen
Zheng, Yiming
Yu, Meng
Deng, Jianwen
Hao, Hongjun
Zhang, Wei
Wang, Zhaoxia
Yuan, Yun
author_facet Jiang, Yanyan
Liu, Yilin
Zhao, Yawen
Zheng, Yiming
Yu, Meng
Deng, Jianwen
Hao, Hongjun
Zhang, Wei
Wang, Zhaoxia
Yuan, Yun
author_sort Jiang, Yanyan
collection PubMed
description OBJECTIVE: This study aimed to investigate mitochondrial changes and the mitochondrial antiviral‐signaling protein (MAVS)‐type I interferon (IFN1) signaling pathway in the muscles of anti‐melanoma differentiation gene 5(MDA5) dermatomyositis (DM) patients. METHODS: Eleven anti‐MDA5 DM and ten antibody‐negative DM patients were included. Muscle biopsies were performed in all patients. Muscle pathology and mitochondrial morphology in particular were compared between two groups. The expression of MDA5, MAVS, interferon (IFN) regulatory factor 7, and IFN‐stimulated gene 15, which are components of the MAVS‐IFN1 signaling pathway, was measured in muscle specimen. The correlation between MAVS expression in muscles and disease phenotypes and muscle pathology were analyzed. RESULTS: Anti‐MDA5 DM showed a significantly lower incidence of the characteristic DM pathology (P < 0.05) than antibody‐negative DM, including perifascicular fiber atrophy, inflammation, and vasculopathy. Mitochondrial abnormalities in anti‐MDA5 patients revealed a high incidence of (8/11,72.7%) and different pattern from that in antibody‐negative DM. MDA5, MAVS, IFN regulatory factor 7, and IFN stimulated gene 15 expression levels in the muscles of anti‐MDA5 DM patients were higher than those of the controls (P < 0.05) but lower than those of antibody‐negative DM patients (P < 0.05). The MAVS levels negatively correlated with manual muscle test 8 scores (r = 0.701, P = 0.016). CONCLUSIONS: Compared to antibody‐negative DM, we presented a different distribution of the mitochondrial pathology and less severe morphology in anti‐MDA5 DM. We also revealed the enhanced but less intensive MAVS‐IFN1 signaling pathway activity in muscles of anti‐MDA5 DM. Such disparity suggested the potentially different mechanism of muscle injury in two DM groups.
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spelling pubmed-79510952021-03-17 Mitochondrial morphology and MAVS‐IFN1 signaling pathway in muscles of anti‐MDA5 dermatomyositis Jiang, Yanyan Liu, Yilin Zhao, Yawen Zheng, Yiming Yu, Meng Deng, Jianwen Hao, Hongjun Zhang, Wei Wang, Zhaoxia Yuan, Yun Ann Clin Transl Neurol Research Articles OBJECTIVE: This study aimed to investigate mitochondrial changes and the mitochondrial antiviral‐signaling protein (MAVS)‐type I interferon (IFN1) signaling pathway in the muscles of anti‐melanoma differentiation gene 5(MDA5) dermatomyositis (DM) patients. METHODS: Eleven anti‐MDA5 DM and ten antibody‐negative DM patients were included. Muscle biopsies were performed in all patients. Muscle pathology and mitochondrial morphology in particular were compared between two groups. The expression of MDA5, MAVS, interferon (IFN) regulatory factor 7, and IFN‐stimulated gene 15, which are components of the MAVS‐IFN1 signaling pathway, was measured in muscle specimen. The correlation between MAVS expression in muscles and disease phenotypes and muscle pathology were analyzed. RESULTS: Anti‐MDA5 DM showed a significantly lower incidence of the characteristic DM pathology (P < 0.05) than antibody‐negative DM, including perifascicular fiber atrophy, inflammation, and vasculopathy. Mitochondrial abnormalities in anti‐MDA5 patients revealed a high incidence of (8/11,72.7%) and different pattern from that in antibody‐negative DM. MDA5, MAVS, IFN regulatory factor 7, and IFN stimulated gene 15 expression levels in the muscles of anti‐MDA5 DM patients were higher than those of the controls (P < 0.05) but lower than those of antibody‐negative DM patients (P < 0.05). The MAVS levels negatively correlated with manual muscle test 8 scores (r = 0.701, P = 0.016). CONCLUSIONS: Compared to antibody‐negative DM, we presented a different distribution of the mitochondrial pathology and less severe morphology in anti‐MDA5 DM. We also revealed the enhanced but less intensive MAVS‐IFN1 signaling pathway activity in muscles of anti‐MDA5 DM. Such disparity suggested the potentially different mechanism of muscle injury in two DM groups. John Wiley and Sons Inc. 2021-02-12 /pmc/articles/PMC7951095/ /pubmed/33576578 http://dx.doi.org/10.1002/acn3.51311 Text en © 2021 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Research Articles
Jiang, Yanyan
Liu, Yilin
Zhao, Yawen
Zheng, Yiming
Yu, Meng
Deng, Jianwen
Hao, Hongjun
Zhang, Wei
Wang, Zhaoxia
Yuan, Yun
Mitochondrial morphology and MAVS‐IFN1 signaling pathway in muscles of anti‐MDA5 dermatomyositis
title Mitochondrial morphology and MAVS‐IFN1 signaling pathway in muscles of anti‐MDA5 dermatomyositis
title_full Mitochondrial morphology and MAVS‐IFN1 signaling pathway in muscles of anti‐MDA5 dermatomyositis
title_fullStr Mitochondrial morphology and MAVS‐IFN1 signaling pathway in muscles of anti‐MDA5 dermatomyositis
title_full_unstemmed Mitochondrial morphology and MAVS‐IFN1 signaling pathway in muscles of anti‐MDA5 dermatomyositis
title_short Mitochondrial morphology and MAVS‐IFN1 signaling pathway in muscles of anti‐MDA5 dermatomyositis
title_sort mitochondrial morphology and mavs‐ifn1 signaling pathway in muscles of anti‐mda5 dermatomyositis
topic Research Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7951095/
https://www.ncbi.nlm.nih.gov/pubmed/33576578
http://dx.doi.org/10.1002/acn3.51311
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