Molecular remodelling of dystrophin in patients with end-stage cardiomyopathies and reversal in patients on assistance-device therapy

Lancet
Matteo VattaJ A Towbin

Abstract

Mutations that lead to disruption of cytoskeletal proteins have been recorded in patients with familial dilated cardiomyopathy. We postulated that changes in cytoskeletal and sarcolemmal proteins provide a final common pathway for dilation and contractile dysfunction in dilated cardiomyopathy. In this study, we investigated the integrity of dystrophin in the myocardium of patients with end-stage heart failure due to ischaemic or dilated cardiomyopathy, and the response to treatment with left-ventricular assistance devices (LVAD). We assessed the expression and integrity of dystrophin in myocardial biopsy samples by immunohistochemistry and western-blot analysis using antibodies against the amino-terminal, carboxyl-terminal, and midrod domains. We took samples from the myocardia of ten controls, ten patients with dilated cardiomyopathy, ten with ischaemic heart disease, and six with dilated cardiomyopathy who underwent placement of a left-ventricular assistance device for progressive refractory heart failure. Immunohistochemical staining identified a disruption to the amino-terminus of dystrophin in 18 of 20 patients with end-stage cardiomyopathy (dilated or ischaemic), whereas staining with antibodies against other domains of d...Continue Reading

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