Loss of plakoglobin immunoreactivity in intercalated discs in arrhythmogenic right ventricular cardiomyopathy: protein mislocalization versus epitope masking
| Autor: | Sebastian Kant, Claudia A. Krusche, Anna Gaertner, Hendrik Milting, Rudolf E. Leube |
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| Rok vydání: | 2015 |
| Předmět: |
Adult
Male 0301 basic medicine Pathology medicine.medical_specialty Adolescent Physiology Plakoglobin 030204 cardiovascular system & hematology Biology Immunofluorescence Desmoglein Right ventricular cardiomyopathy Epitope Epitopes Young Adult 03 medical and health sciences 0302 clinical medicine Desmosome Physiology (medical) medicine Animals Humans Arrhythmogenic Right Ventricular Dysplasia Aged Mice Knockout medicine.diagnostic_test Myocardium Desmosomes Middle Aged medicine.disease Arrhythmogenic right ventricular dysplasia Disease Models Animal Phenotype 030104 developmental biology medicine.anatomical_structure Desmoplakins Female gamma Catenin Cardiology and Cardiovascular Medicine Intercalated disc |
| Zdroj: | Cardiovascular Research. 109:260-271 |
| ISSN: | 1755-3245 0008-6363 |
| DOI: | 10.1093/cvr/cvv270 |
| Popis: | Aims Examine the relevance and cause of reduced plakoglobin immunofluorescence in intercalated discs for arrhythmogenic right ventricular cardiomyopathy (ARVC) and ARVC-like disease in mouse and human. Methods and Results Normalized semiquantitative immunofluorescence 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. |
| Databáze: | OpenAIRE |
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