Mouse models for cardiomyopathy research

Cardiomyopathy refers to a family of inherited or acquired systemic diseases that primarily affect cardiac muscle. Cardiomyopathies can be divided into four main subtypes, based on morphological and physiological defects within the cardiac muscle itself, which include: (1) dilated cardiomyopathy, (2) hypertrophic cardiomyopathy, (3) restrictive cardiomyopathy and (4) arrhythmogenic right ventricular dysplasia/cardiomyopathy. Genetics have been shown to play an increasingly important role in the onset of cardiomyopathies. As a result, the mouse has emerged as the most used animal model for cardiomyopathy research, largely based on the availabilities of genetic technologies to manipulate the mouse genome. Mouse models have given the cardiovascular field significant insights into the molecular mechanisms and genetic factors underlying the etiology and pathogenesis of various cardiomyopathies. This review will first, discuss genetic approaches to generate mouse models as well as highlight certain mouse models which have exploited gene knockout and transgenic expression of mutant proteins associated with human cardiomyopathies, to study mechanisms involved in the pathogenesis of these various subtypes of cardiomyopathies.