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Characterization of transgenic mouse models for Arhythmogenic Right Ventricular Cardiomyopathy

Arhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease characterized by progressive loss of cardiomyocytes and fibro-fatty replacement of the heart muscle, with alteration of electrical rhythm of the heart and sudden death. To study molecular mechanisms involved in pathogenesis of ARVC, different lines of transgenic mice overexpressing normal or mutated human desmoglein-2, were characterized. The alteration of the myocardium in transgenic mice was demonstrated through histological analysis, which has permitted us to observe fibrosis (Azan Mallory staining) and calcification (Van Kossa staining). During these experiments were observed also some areas of probable fatty replacement, which was confirmed by specific Oil-Red-O staining that was performed on cardiac tissue. In order to study the molecular pathogenesis of the disease, western blot assays were performed. Analysis showed an increase in β-catenin in myocardium of transgenic mice and the up regulation of the expression of Cyclin D1, a protein that regulates the cell cycle and which is probably involved in fibrogenesis. In transgenic mice was also noticed a decrease in Plakoglobin, like observed in studies on patients with ARVC, confirming the parallel between the human phenotype and the one induced in transgenic mice.

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1 Abstract Arrhythmogenic right ventricular cardiomyopathy (AR VC) is a myocardial disease characterized by progressive loss of cardiomyocytes and fibro-fatty replacement of the heart muscle, with alteration of electrical rhythm of the heart and sudden death. To study molecular mechanisms involved in pa thogenesis of ARVC, different lines of transgenic mice overexpressing n ormal or mutated human desmoglein-2, were characterized. The alteration of the myocardium in transgenic mice was demonstrated through histologic al analysis, which has permitted us to observe fibrosis (Azan Mallory stai ning) and calcification (Van Kossa staining). During these experiments were obse rved also some areas of probable fatty replacement, which was confirmed by specific Oil-Red-O staining that was performed on cardiac tissue. In order to s tudy the molecular pathogenesis of the disease, western blot assays we re performed. Analysis showed an increase in β-catenin in myocardium of tr ansgenic mice and the up regulation of the expression of Cyclin D1, a protei n that regulates the cell cycle and which is probably involved in fibrogenesis. In transgenic mice was also noticed a decrease in Plakoglobin, like observed in studies on patients with ARVC, confirming the parallel between the human phe notype and the one induced in transgenic mice.

Laurea liv.I

Facoltà: Medicina e Chirurgia

Autore: Marco Maggioni Contatta »

Composta da 35 pagine.

 

Questa tesi ha raggiunto 110 click dal 14/01/2011.

 

Consultata integralmente una volta.

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