Molecular mechanism of ventricular trabeculation/compaction and the pathogenesis of the left ventricular noncompaction cardiomyopathy (LVNC)

Wei Zhang1, Hanying Chen, Xiuxia Qu2, Ching-Pin Chang, Weinian Shou3
1Riley Heart Research Center, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
2Indiana University, Bloomington
3Cardiac Developmental Biology

Tóm tắt

AbstractVentricular 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. © 2013 Wiley Periodicals, Inc.

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