**3. Animal models of LVNC**

LVNC is an overlap disorder and it appears that any of these "final common pathways" can be involved depending on the specific form of LVNC [64]. Combining information about disease-causing genes with murine models is crucial in identifying pathways involved in ventricular noncompaction. For instance, Barth syndrome is caused by tafazzin mutations, and tafazzin knockdown mice were engineered using a short-hairpin RNA-inducible transgenic approach. These mice demonstrated hypertrabeculation and noncompaction, and the knockdown mice died prenatally at E12.5–14.5 [65]. New powerful cutting-edge gene-editing technologies using transcription activator-like effector nucleases (TALENs) and clustered regularly interspaced short palindromic repeats-CRISPR-associated protein 9 (CRISPR-Cas9) have been used for modeling LVNC [66–69]. Patient-specific induced pluripotent stem cells (iPSCs) derived cardiomyocytes have emerged as a useful tool for investigating pathological mechanisms of many cardiovascular diseases including LVNC [70, 71].

*DOI: http://dx.doi.org/10.5772/intechopen.101085* **Gene Animal model Signaling Ref #** FKBP12 KO mouse Decrease in Notch1 activity, increase in BMP10 [79]

*Left Ventricular Noncompaction Cardiomyopathy: From Clinical Features to Animal Modeling*


**Table 1.** *Animal models of LVNC.*

Several signaling pathways such as the Dll4-NOTCH [72], MIB1 [73], BMP [74], and TGF-β [71] have been demonstrated to regulate myocardial trabecular compaction as well as to be involved in the development of LVNC. While zebrafish and *Drosophila* have been used to study LVNC in addition to patient-specific iPSCs derived cardiomyocytes, mice are more commonly used to genetically engineer LVNC phenotypes as shown in **Table 1** [75, 76].
