Little Mice with Big Hearts: Finding the Molecular Basis for Dilated Cardiomyopathy

摘要: Dilated cardiomyopathy (DCM) is characterized by increased ventricular chamber size and dimension coupled with loss of contractile function, myofibril organization 13-adrenergic responsiveness. [1] Typical microscopic features include high incidence myocyte hypertrophy apoptosis, [2, 3, 4] varying degrees fibrosis the presence lymphocytes in interstitial space [5]. Clinically, disease displays heart failure symptoms consistent New York Heart Association (NYHA) functional class III or IV at time diagnosis [6] remains principal indication for transplantation both adults pediatric cardiology [6, 7, 8]. The clinical treatment human DCM a substantial economic burden estimated to cost $10 $40 billion annually United States alone [9]. First foremost terms pathologic consequences, distinguished profound myocardial function. degeneration structure function cardiomyocytes observed dilation less amenable toin vitroanalysis compared hypertrophie changes associated enhanced enlarged cardiomyocyte that are readily apparent analyses cultured cells. Cardiomyocyte remodeling results from combination physical stresses signal transduction leading structural entire heart. This combinatorial environment best reproducedin vivoas evidenced emergence transgenic gene targeted animal models display characteristic DCM. These useful study temporal progression pathogenic alterations occur molecular, cellular organ levels. Many genetic mouse have emerged over past decade, many which remodeling, whether not scope this review, however, concentrate on those multiple characteristics