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. |