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Central role for GSK3β in the pathogenesis of arrhythmogenic cardiomyopathy
Open accessAbstract
Arrhythmogenic cardiomyopathy (ACM) is characterized by redistribution of junctional proteins, arrhythmias, and progressive myocardial injury. We previously reported that SB216763 (SB2), annotated as a GSK3β inhibitor, reverses disease phenotypes in a zebrafish model of ACM. Here, we show that SB2 prevents myocyte injury and cardiac dysfunction in vivo in two murine models of ACM at baseline and in response to exercise. SB2-treated mice with desmosome mutations showed improvements in ventricular ectopy and myocardial fibrosis/inflammation as compared with vehicle-treated (Veh-treated) mice. GSK3β inhibition improved left ventricle function and survival in sedentary and exercised Dsg2 mut/mut mice compared with Veh-treated Dsg2 mut/mut mice and normalized intercalated disc (ID) protein distribution in both mutant mice. GSK3β showed diffuse cytoplasmic localization in control myocytes but ID redistribution in ACM mice. Identical GSK3β redistribution is present in ACM patient myocardium but not in normal hearts or other cardiomyopathies. SB2 reduced total GSK3β protein levels but not phosphorylated Ser 9–GSK3β in ACM mice. Constitutively active GSK3β worsens ACM in mutant mice, while GSK3β shRNA silencing in ACM cardiomyocytes prevents abnormal ID protein distribution. These results highlight a central role for GSKβ in the complex phenotype of ACM and provide further evidence that pharmacologic GSKβ inhibition improves cardiomyopathies due to desmosome mutations.
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“This model exhibits progressive myocardial inflammation, fibrotic remodeling, and ventricular dysfunction and arrhythmias.”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
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“findings from this model demonstrate that myocardial inflammation arises intrinsically (i.e., from cardiomyocytes) due to the presence of a desmosomal mutation, rather than solely from infiltrative immune cells, supporting the concept that inflammation is a primary driver of disease progression and an emerging therapeutic target in ACM.”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
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“Uniquely, GSK3β localization at the intercalated disc (ID) was observed in ACM hearts. This finding was in stark contrast to other inflammatory heart diseases, such as giant cell myocarditis and cardiac sarcoidosis, where GSK3β localization was predominantly cytoplasmic [60].”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
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“Aberrant GSK3β activity in ACM is also linked to the deleterious activation of NFκB [11,12,60].”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
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“Together, the suppression of Wnt and overactivation of NFκB drive pro-inflammatory, adipogenic, and fibrotic gene expression programs [60].”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
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“Pharmacological inhibition of GSK3β (e.g., SB216763) prevented myocyte injury and cardiac dysfunction in two murine models of ACM (Dsg2mut/mut and JUP2157del2) at rest and in response to exercise, where SB216763-treated mice demonstrated improvements in ventricular ectopy and function, myocardial fibrosis and inflammation, and normalization of ID proteins, and, most importantly, increased survival”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
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“The germline Dsg2mut/mut mouse model developed by Chelko SP and colleagues represents a more genetically relevant system for studying ACM pathogenesis, as the Dsg2 mutation is germline, mirroring the pattern of inheritance observed in patients [12,60].”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
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“uniquely distinguishing features have been noted in ACM hearts, such as loss of junctional plakoglobin (JUP), Cx43 [73], and SAP97 [74] at the ID, in conjunction with GSK3β redistribution to the ID [60], demonstrating that desmosomal disruption imparts uniquely specific pathological characteristics in ACM.”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
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“Dysregulation of GSK3β, involved in the suppression of the Wnt/β-catenin pathway, has also been implicated in ACM pathogenesis, particularly in the promotion of fibrosis and inflammation, and is effectively inhibited by SB216763 in preclinical ACM models [60,74,116,117].”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
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“In Dsg2mut/mut mice, myocardial levels of IL-1β, IFN-γ, IL-12, LIX (CXCL5), and Osteopontin (OPN) are substantially elevated.”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
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“Multiple pro-inflammatory cytokines and chemokines have been shown to be upregulated early in ACM pathogenesis.”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
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“Notably, LIX and OPN levels show a significant inverse correlation with cardiac function and a positive correlation with myocardial fibrosis [60]”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [60]
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Metadata
- Authors
- Chelko, S. P., Asimaki, A., Andersen, P., Bedja, D., Amat-Alarcon, N., DeMazumder, D., Jasti, R., MacRae, C. A., Leber, R., Kleber, A. G., Saffitz, J. E., & Judge, D. P.
- Affiliations
- Johns Hopkins University (US)
- Beth Israel Deaconess Medical Center (US)
- Department of Medicine/Cardiology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
- Brigham and Women's Hospital (US)
- Schiller AG, Research and Development, Baar, Switzerland.
- Department of Pathology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts, USA.
- Corresponding author
- Saffitz, Jeffrey E.
- Judge, Daniel P.
- ORCID
- Venue
- JCI Insight
- Published
- 2016-04-21
- Volume
- 1
- Issue
- 5
- ISSN
- 2379-3708 (ISSN-L)
- PMC
- PMC4861310
- OpenAlex
- W2341491381
- Cited by
- 180
- References
- 39
- Citations by year
- 2026: 10 · 2025: 27 · 2024: 16 · 2023: 23 · 2022: 12 · 2021: 30 · 2020: 21 · 2019: 14 · 2018: 12 · 2017: 13 · 2016: 2
- FWCI
- 11.26
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- JCI Insight (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- American Heart Association
- University of Miami
- Hartwell Foundation
- National Institutes of Health (R24OD017870)
- Leonard M. Miller School of Medicine
- National Heart, Lung, and Blood Institute (RC1HL100110, K99HL130662, R01HL102361)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Keywords
- arrhythmogenic cardiomyopathy, GSK3β, SB216763, GSK-3β inhibition, desmoglein 2, ventricular ectopy, myocardial fibrosis, exercise-induced cardiomyopathy, cardiac dysfunction, zebrafish model, mouse model, cardiomyocyte injury
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