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Publication : Loss of plakoglobin immunoreactivity in intercalated discs in arrhythmogenic right ventricular cardiomyopathy: protein mislocalization versus epitope masking.

First Author  Kant S Year  2016
Journal  Cardiovasc Res Volume  109
Issue  2 Pages  260-71
PubMed ID  26676851 Mgi Jnum  J:252640
Mgi Id  MGI:6107365 Doi  10.1093/cvr/cvv270
Citation  Kant S, et al. (2016) Loss of plakoglobin immunoreactivity in intercalated discs in arrhythmogenic right ventricular cardiomyopathy: protein mislocalization versus epitope masking. Cardiovasc Res 109(2):260-71
abstractText  AIMS: To examine the relevance and cause of reduced plakoglobin IF in intercalated discs for arrhythmogenic right ventricular cardiomyopathy (ARVC) and ARVC-like disease in mouse and human. METHODS AND RESULTS: Normalized semi-quantitative IF measurements were performed in a standardized format in desmoglein 2-mutant mice with an ARVC-like phenotype (n = 6) and in cardiac biopsies from humans with ARVC and non-ARVC heart disease (n = 10). Reduced plakoglobin staining was detectable in ARVC only with one antibody directed against a defined epitope but not with three other antibodies reacting with different epitopes of plakoglobin. CONCLUSIONS: Reduced plakoglobin staining in intercalated discs of heart tissue from human ARVC patients and in a murine ARVC model is caused by alterations in epitope accessibility and not by protein relocalization.
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