Genetic predisposition to immune dysregulation and extracellular matrix remodeling in cardiac arrhythmia reveals potential mediation by SPP1+ macrophages

IntroductionCardiac arrhythmia frequently co-presents with structural abnormalities such as cardiomyopathy and myocardial fibrosis, creating a bidirectional relationship where electrical disturbances and structural remodeling exacerbate each other. Current genetic studies focus on ion channel varian...

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Main Authors: Jie-Yuan Jin, Shuai Guo, Yao Deng, Ya-Qin Chen, Chen Liang, Yu-Jie Jiang, Wang Zhao, Rong Xiang
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-08-01
Series:Frontiers in Cell and Developmental Biology
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Online Access:https://www.frontiersin.org/articles/10.3389/fcell.2025.1611663/full
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author Jie-Yuan Jin
Jie-Yuan Jin
Shuai Guo
Yao Deng
Ya-Qin Chen
Chen Liang
Yu-Jie Jiang
Wang Zhao
Rong Xiang
author_facet Jie-Yuan Jin
Jie-Yuan Jin
Shuai Guo
Yao Deng
Ya-Qin Chen
Chen Liang
Yu-Jie Jiang
Wang Zhao
Rong Xiang
author_sort Jie-Yuan Jin
collection DOAJ
description IntroductionCardiac arrhythmia frequently co-presents with structural abnormalities such as cardiomyopathy and myocardial fibrosis, creating a bidirectional relationship where electrical disturbances and structural remodeling exacerbate each other. Current genetic studies focus on ion channel variants, which explain part of the etiology. Molecular mechanisms underlying arrhythmias pathogenesis and its progression warrant further investigation.MethodsWe performed whole-exome sequencing on 50 arrhythmia patients (21 females, 29 males), predominantly with early-onset disease (94% ≤ 35 years). We focused on exonic deleterious mutations that are rare in healthy populations. The identified recurrently mutated (r.m.) genes were analyzed using protein-protein interaction networks and gene ontology enrichment for functional modules. These genomic insights were integrated with single-cell data (7 arrhythmias, 5 controls) to examine cell-type-specific gene expression changes, with particular focus on SPP1+ macrophage states.ResultsWe identified 132 r.m. genes present in ≥30% of patients in our cohort, with significant functional module enrichment in immune regulation, tissue homeostasis, extracellular matrix, and vesicle transport pathways. Single-cell analysis of 37,675 cells revealed conserved transcriptional signatures across cell types, characterized by enhanced cytokine responses and pro-fibrogenic programs. We discovered genetic determinants potentially underlying SPP1+ macrophage activation in arrhythmic hearts—a known mediator implicated in both inflammatory processes and fibrotic remodeling. Age-specific associations included ADAMTS7 mutations in very early-onset cases (≤20years; OR = 9.71 [2.38–47.74], P-value <0.001), while gender-specific variants included SLC9B1 (P-value = 0.017) exclusively in females. Additionally, OTOA mutations were associated with both relatively late onset (>20years; OR = 0.17 [0.04–0.68], P-value = 0.009) and female predominance (OR = 3.41 [0.92–13.58], P-value = 0.045).ConclusionOur exploratory analysis reveals how genetic variants may predispose arrhythmia patients to inflammatory and fibrotic processes. These findings may help guide future research into the molecular mechanisms underlying arrhythmia progression to structural heart disease and identify candidate pathways for therapeutic investigation.
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spelling doaj-art-a5733d8a8ca24fd98866558621ce0ecb2025-08-20T04:03:12ZengFrontiers Media S.A.Frontiers in Cell and Developmental Biology2296-634X2025-08-011310.3389/fcell.2025.16116631611663Genetic predisposition to immune dysregulation and extracellular matrix remodeling in cardiac arrhythmia reveals potential mediation by SPP1+ macrophagesJie-Yuan Jin0Jie-Yuan Jin1Shuai Guo2Yao Deng3Ya-Qin Chen4Chen Liang5Yu-Jie Jiang6Wang Zhao7Rong Xiang8School of Medicine, Shaoxing University, Shaoxing, ChinaSchool of Life Sciences, Central South University, Changsha, ChinaSchool of Life Sciences, Central South University, Changsha, ChinaDepartment of Cardiovascular Surgery, Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Cardiovascular Medicine, the Second Xiangya Hospital, Central South University, Changsha, ChinaCenter for Medical Genetics, Jiangmen Maternal & Child Healthcare Hospital, Jiangmen, ChinaSchool of Life Sciences, Central South University, Changsha, ChinaDepartment of Cardiovascular Medicine, the Second Xiangya Hospital, Central South University, Changsha, ChinaSchool of Life Sciences, Central South University, Changsha, ChinaIntroductionCardiac arrhythmia frequently co-presents with structural abnormalities such as cardiomyopathy and myocardial fibrosis, creating a bidirectional relationship where electrical disturbances and structural remodeling exacerbate each other. Current genetic studies focus on ion channel variants, which explain part of the etiology. Molecular mechanisms underlying arrhythmias pathogenesis and its progression warrant further investigation.MethodsWe performed whole-exome sequencing on 50 arrhythmia patients (21 females, 29 males), predominantly with early-onset disease (94% ≤ 35 years). We focused on exonic deleterious mutations that are rare in healthy populations. The identified recurrently mutated (r.m.) genes were analyzed using protein-protein interaction networks and gene ontology enrichment for functional modules. These genomic insights were integrated with single-cell data (7 arrhythmias, 5 controls) to examine cell-type-specific gene expression changes, with particular focus on SPP1+ macrophage states.ResultsWe identified 132 r.m. genes present in ≥30% of patients in our cohort, with significant functional module enrichment in immune regulation, tissue homeostasis, extracellular matrix, and vesicle transport pathways. Single-cell analysis of 37,675 cells revealed conserved transcriptional signatures across cell types, characterized by enhanced cytokine responses and pro-fibrogenic programs. We discovered genetic determinants potentially underlying SPP1+ macrophage activation in arrhythmic hearts—a known mediator implicated in both inflammatory processes and fibrotic remodeling. Age-specific associations included ADAMTS7 mutations in very early-onset cases (≤20years; OR = 9.71 [2.38–47.74], P-value <0.001), while gender-specific variants included SLC9B1 (P-value = 0.017) exclusively in females. Additionally, OTOA mutations were associated with both relatively late onset (>20years; OR = 0.17 [0.04–0.68], P-value = 0.009) and female predominance (OR = 3.41 [0.92–13.58], P-value = 0.045).ConclusionOur exploratory analysis reveals how genetic variants may predispose arrhythmia patients to inflammatory and fibrotic processes. These findings may help guide future research into the molecular mechanisms underlying arrhythmia progression to structural heart disease and identify candidate pathways for therapeutic investigation.https://www.frontiersin.org/articles/10.3389/fcell.2025.1611663/fullcardiac arrhythmiaswhole-exome sequencing (WES)single-cell transcriptomicsSPP1+ macrophageimmune dysregulationextracellular matrix remodeling
spellingShingle Jie-Yuan Jin
Jie-Yuan Jin
Shuai Guo
Yao Deng
Ya-Qin Chen
Chen Liang
Yu-Jie Jiang
Wang Zhao
Rong Xiang
Genetic predisposition to immune dysregulation and extracellular matrix remodeling in cardiac arrhythmia reveals potential mediation by SPP1+ macrophages
Frontiers in Cell and Developmental Biology
cardiac arrhythmias
whole-exome sequencing (WES)
single-cell transcriptomics
SPP1+ macrophage
immune dysregulation
extracellular matrix remodeling
title Genetic predisposition to immune dysregulation and extracellular matrix remodeling in cardiac arrhythmia reveals potential mediation by SPP1+ macrophages
title_full Genetic predisposition to immune dysregulation and extracellular matrix remodeling in cardiac arrhythmia reveals potential mediation by SPP1+ macrophages
title_fullStr Genetic predisposition to immune dysregulation and extracellular matrix remodeling in cardiac arrhythmia reveals potential mediation by SPP1+ macrophages
title_full_unstemmed Genetic predisposition to immune dysregulation and extracellular matrix remodeling in cardiac arrhythmia reveals potential mediation by SPP1+ macrophages
title_short Genetic predisposition to immune dysregulation and extracellular matrix remodeling in cardiac arrhythmia reveals potential mediation by SPP1+ macrophages
title_sort genetic predisposition to immune dysregulation and extracellular matrix remodeling in cardiac arrhythmia reveals potential mediation by spp1 macrophages
topic cardiac arrhythmias
whole-exome sequencing (WES)
single-cell transcriptomics
SPP1+ macrophage
immune dysregulation
extracellular matrix remodeling
url https://www.frontiersin.org/articles/10.3389/fcell.2025.1611663/full
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