How can we use stem cell-derived cardiomyocytes to understand the involvement of energetic metabolism in alterations of cardiac function?

Mutations in the mitochondrial-DNA or mitochondria related nuclear-encoded-DNA lead to various multisystemic disorders collectively termed mitochondrial diseases. One three cases of disease affects heart muscle, which is called cardiomyopathy (MCM) and associated with hypertrophic, dilated, noncompact cardiomyopathy. The an organ high energy demand, occupy 30%–40% its cardiomyocyte-cell volume. Mitochondrial dysfunction leads depletion has detrimental effects on cardiac performance. However, development progression context nuclear DNA mutations, remains incompletely understood. system induced pluripotent stem cell (iPSC)-derived cardiomyocytes (CM) excellent platform study MCM since unique genetic identity their donors enables a robust recapitulation predicted phenotypes dish patient-specific level. Here, we focus recent insights into studied by iPSC-CM further discuss research gaps advances metabolic maturation iPSC-CM, crucial for develop novel therapeutic strategies.