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Am J Med Genet C Semin Med Genet. 2013 Aug;163C(3):144-56. doi: 10.1002/ajmg.c.31369. Epub 2013 Jul 10.

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

Author information

1
Riley Heart Research Center, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

Abstract

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.

PMID:
23843320
PMCID:
PMC3725649
DOI:
10.1002/ajmg.c.31369
[Indexed for MEDLINE]
Free PMC Article

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