*2.2.2 Dilated cardiomyopathy animal models*

Animal models of DCM mostly resemble human mutations in genes encoding cytoskeletal, sarcomeric, and Z-disk proteins and present with ventricular dilation and thinning of the ventricular walls correlated with loss of heart muscle mass. In addition, functional changes in non-myocytes induce fibrotic scars that


#### **Table 2.**

*Animal models of hypertrophic cardiomyopathy [51–58, 60–81].*

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 been explored (**Table 2**).


**7**

**Table 5.**

**Table 4.**

*Animal Models of Cardiomyopathies*

*DOI: http://dx.doi.org/10.5772/intechopen.89033*

*2.2.3 Restrictive cardiomyopathy animal models*

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

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

troponins, myosin and MYPN (summarized in **Table 3**).

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

#### **Table 3.**

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