Enhanced Myocardial Deposition of DSG2-Reactive IgG in a Patient with Arrhythmogenic Right Ventricular Cardiomyopathy
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited myocardial disease characterized by desmosomal disruption, fibrofatty replacement, and ventricular arrhythmias. Although autoantibodies against desmoglein-2 (DSG2) are implicated in ARVC, their myocardial localization remains unproven. We investigated DSG2-reactive IgG in myocardial tissue from a patient with ARVC and compared the findings with non-failing donor myocardium. Immunofluorescence and confocal microscopy revealed reduced, discontinuous DSG2 expression and altered desmoplakin and plakoglobin distribution. IgG colocalized more extensively with DSG2 in ARVC than in control myocardium, consistent with elevated serum anti-DSG2 autoantibodies. These findings provide tissue-level evidence of the spatial co-occurrence of tissue-bound IgG and DSG2 in ARVC and support further investigation of autoimmune mechanisms in disease progression.
Authors
- Przemysław Leszek (ORCID: https://orcid.org/0000-0003-4529-2687)
- Karolina Borowiec (ORCID: https://orcid.org/0000-0002-0719-128X)
- Olgierd Woźniak (ORCID: https://orcid.org/0000-0001-6781-8198)
- Ewa Warchol Celinska (ORCID: https://orcid.org/0000-0002-6757-2527)
- Mirosław Kowalski (ORCID: https://orcid.org/0000-0002-7039-7723)
- Jarosław Walory (ORCID: https://orcid.org/0000-0003-2352-2621)
- Kamila Maliszewska‐Olejniczak (ORCID: https://orcid.org/0000-0002-1521-199X)
- Łukasz Nowotka
- Elżbieta Katarzyna Biernacka (ORCID: https://orcid.org/0000-0003-3215-5085)
- Katarzyna Wiktorska (ORCID: https://orcid.org/0000-0002-2731-9535)
- Patryk Krzemiński (ORCID: https://orcid.org/0000-0002-0526-9135)
- Jolanta Krzysztoń-Russjan (ORCID: https://orcid.org/0000-0001-7200-6178)
- Ilona Minota (ORCID: https://orcid.org/0000-0002-3106-5427)
- Irena Wadas
Institutions
- Warsaw University of Life Sciences (PL)
- Institute of Cardiology (PL)
Publication Details
- Journal
- International Journal of Molecular Sciences
- Published
- 2026-11-11
- DOI
- https://doi.org/10.3390/ijms272210087
- Primary Topic
- Cardiovascular Effects of Exercise
- Type
- article
- Field-Weighted Citation Impact
- 0.00