*2.2.3 Restrictive cardiomyopathy animal models*

RCM is the least common but most lethal form of cardiomyopathy where impaired ventricular relaxation due to increased stiffness of the myocardium and pressure in the ventricles overcomes the changes in myofibrillar arrangement and cardiomyocyte gross abnormalities [113]. Animal models carrying human RCMassociated mutations have also been generated to mimic human RCM phenotype. These mutations are identified mainly in sarcomeric protein-encoding genes such as troponins, myosin and MYPN (summarized in **Table 3**).


#### **Table 4.**

*Animal Models in Medicine and Biology*

been explored (**Table 2**).

stiffen the heart tissue and impede normal cardiomyocyte contractility. Novel DCM mechanisms such as impaired Z-disk assembly, sensitivity to apoptosis and abnormalities in myofibrillogenesis under metabolic stress, protein folding, inhibition of protein aggregation, and degradation of misfolded proteins have

**6**

**Table 3.**

*Animal models of dilated cardiomyopathy [82–112].*

*Animal models of restrictive cardiomyopathy [67, 114–117].*


#### **Table 5.**

*Animal models of arrhythmogenic ventricular cardiomyopathy [119–139].*


#### **Table 6.**

*Animal models of left ventricular noncompaction cardiomyopathy [141–159].*
