Prospects for the Use of Cell Cultures in Modeling Myocardial Diseases: Hypertrophic Cardiomyopathy

Abstract

The use of various model organisms has made a huge contribution to understanding the causes and mechanisms of disease development and the study of pathological processes occurring during the development of diseases of the human cardiovascular system, and, in particular, hypertrophic cardiomyopathy (HCM). The optimal solution in the study of primary molecular disturbances is the use of cellular models such as induced pluripotent stem cells (iPSCs), primary rodent cardiomyocytes (CMs), and immortalized lines. In this review, we have focused on the most commonly used cell models, including freshly isolated adult and neonatal rodent CMs, and on the commercially available immortalized cell lines (HL-1, AC16, and H9c2). In order to assess the adequacy of these lines as CM models for studying human myocardial pathologies, a comparative analysis of phenotypic characteristics (morphology, metabolism, calcium homeostasis, etc.) and the nuances of practical use (availability, response to hypertrophic inducers, transfection, etc.) was carried out. The latest published data on the use of these models to assess the pathogenicity of HCM-associated mutations, as well as to screen the effectiveness of developed therapeutic drugs, are also summarized.