Fig. 7

Fig. 7 Summary of the pathological model for R9C mutation. PLN^R9C mutation leads to increased Ca²⁺ levels, contractility, and a blunted β-adrenergic response in the heart, consistent with the observations in 3 dpf larvae. The chronic inotropic stimulation caused by R9C, and the energetic demand exerts a pathological effect displaying ventricular dilation, systolic dysfunction, bradycardia and negative lusitropy, as observed in 9 dpf larvae. A reduced antioxidant capacity may alter the redox balance in line with the observation in 9 dpf treated with NAC . Asterisks indicate the findigs shown in this work; the mitochondrial changes are a working model (ETC, electron transport chain; MCU, mitochondrial Ca²⁺ uniporter; ROS, reactive oxygen species).