Evidence map›Paper›PMID 41876697›Full record

ArticlePediatric research2026

Integrated transcriptomics and proteomics analysis reveal potential target in pediatric primary restrictive cardiomyopathy.

Xihang Fu, Jie Liu, Qing Guo, Lin Wang, Jing Wu, Zubo Wu, Yanlin Xing, Peng Zhu, Nianguo Dong, Jiawei Shi and 1 more

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Article in Pediatric research, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 · Who and what money

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11 authors.

Xihang Fu *Key Laboratory of Environment and Health, Ministry of Education & Ministry of Environmental Protection, Department of Epidemiology and Biostatistics, School of Public Health, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Jie Liu *Department of Pediatrics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Qing GuoDepartment of Pediatrics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Lin WangDepartment of Pediatrics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Jing WuKey Laboratory of Environment and Health, Ministry of Education & Ministry of Environmental Protection, Department of Epidemiology and Biostatistics, School of Public Health, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Zubo WuDepartment of Pediatrics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Yanlin XingDepartment of Pediatrics, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China.
Peng ZhuDepartment of cardiovascular surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Nianguo DongDepartment of cardiovascular surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
Jiawei ShiDepartment of cardiovascular surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China. shijiawei@21cn.com.
Hua PengDepartment of Pediatrics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China. 2006xh0830@hust.edu.cn.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThe heterogeneous etiology and limited therapeutic options of pediatric restrictive cardiomyopathy (RCM)underscore the urgent need to elucidate its molecular mechanisms and identify potential treatment targets.

methodsWe performed integrated transcriptomic and proteomic analyses on myocardial tissues from 7 pediatric RCM patients and 3 control donors. Differentially expressed genes (DEG) were detected, with log

resultsMulti-omics integration revealed 23 consistently dysregulated genes/proteins central to RCM pathogenesis (FDR adjusted P < 0.05). RNA validation confirmed significant expression changes in most hub genes, while protein-level assays demonstrated marked downregulation of CKB, PGAM2, and TPM2 in RCM myocardium (P < 0.05). Functional enrichment analysis highlighted the involvement of these molecules in critical pathways, including muscle contraction, sarcomere organization, and extracellular matrix remodeling. Drug prediction analysis identified several repurposed candidates, including phenytoin, diazepam, and paricalcitol, which may target these aberrant pathways.

conclusionsThis study elucidates the fundamental molecular mechanisms underlying muscle contraction and energy metabolism in RCM, while simultaneously translating these insights into clinically actionable strategies. Specifically, it identifies the downregulation of key proteins as potential biomarkers and proposes drug repurposing strategies informed by pathway analysis. These findings offer a novel perspective on the pathophysiology of RCM and establish an evidence-based foundation for the development of targeted diagnostic and therapeutic tools. IMPACT: This study offers a novel perspective on the mechanisms underlying pediatric primary restrictive cardiomyopathy, specifically highlighting abnormalities in muscle regulation and the myocardial extracellular matrix. Additionally, the predicted therapeutic targets for pediatric primary restrictive cardiomyopathy indicate the new directions for their clinical management except for the treatment of advanced heart failure.

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