Genetically engineered mouse models demonstrate that disruptions in Notch, BMP10, and noncanonical Wnt/PCP signaling pathways contribute to the pathogenesis of left ventricular noncompaction.
Ventricular trabeculation and compaction are two of the many essential steps for generating a functionally competent ventricular wall. A significant reduction in trabeculation is usually associated with ventricular compact zone deficiencies (hypoplastic wall), which commonly leads to embryonic heart failure and early embryonic lethality. In contrast, hypertrabeculation and lack of ventricular wall compaction (noncompaction) are closely related defects in cardiac embryogenesis associated with left ventricular noncompaction (LVNC), a genetically heterogenous disorder. Here we review recent findings through summarizing several genetically engineered mouse models that have defects in cardiac trabeculation and compaction.
Zhang et al. (Wed,) conducted a review in Left ventricular noncompaction cardiomyopathy (LVNC). Genetically engineered mouse models demonstrate that disruptions in Notch, BMP10, and noncanonical Wnt/PCP signaling pathways contribute to the pathogenesis of left ventricular noncompaction.
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