Ultrastructural Changes in Cardiac Myocytes from Boxer Dogs with Arrhythmogenic Right Ventricular Cardiomyopathy

Overview

Journal J Vet Cardiol

Publisher Elsevier

Specialties Cardiology & Vascular Diseases
Veterinary Medicine

Date 2011 Jun 4

PMID 21636338

Citations 20

Authors

Eva M Oxford

Charles G Danko

Bruce G Kornreich

Karen Maass

Shari A Hemsley

Dima Raskolnikov

Philip R Fox

Mario Delmar

N Sydney Moise

Affiliations

Soon will be listed here.

Abstract

Objectives: We sought to quantify the number and length of desmosomes, gap junctions, and adherens junctions in arrhythmogenic right ventricular cardiomyopathy (ARVC) and non-ARVC dogs, and to determine if ultrastructural changes existed.

Animals: Hearts from 8 Boxer dogs afflicted with histopathologically confirmed ARVC and 6 dogs without ARVC were studied.

Methods: Quantitative transmission electron microscopy (TEM) and Western blot semi-quantification of α-actinin were used to study the intercalated disc and sarcomere of the right and left ventricles.

Results: When ARVC dogs were compared to non-ARVC dogs reductions in the number of desmosomes (P = 0.04), adherens junctions (P = 0.04) and gap junctions (P = 0.02) were found. The number of gap junctions (P = 0.04) and adherens junctions (P = 0.04) also were reduced in the left ventricle, while the number of desmosomes was not (P = 0.88). A decrease in the length of desmosomal complexes within LV samples (P = 0.04) was found. These findings suggested disruption of proteins providing attachment of the cytoskeleton to the intercalated disc. Immunoblotting did not demonstrate a quantitative reduction in the amount of α-actinin in ARVC afflicted samples. All Boxers with ARVC demonstrated the presence of electron dense material originating from the Z band and extending into the sarcomere, apparently at the expense of the cytoskeletal structure.

Conclusions: These results emphasize the importance of structural integrity of the intercalated disc in the pathogenesis of ARVC. In addition, observed abnormalities in sarcomeric structure suggest a novel link between ARVC and the actin-myosin contractile apparatus.

Citing Articles

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Tmem65 is critical for the structure and function of the intercalated discs in mouse hearts.

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Immunofluorescent Localization of Plakoglobin Is Altered in Endomyocardial Biopsy Samples from Dogs with Clinically Relevant Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC).

Oxford E, Pariaut R, Tursi M, Fox P, Santilli R Vet Sci. 2021; 8(11).

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