Analysis of ventricular hypertrabeculation and noncompaction using genetically engineered mouse models

Pediatr Cardiol. 2009 Jul;30(5):626-34. doi: 10.1007/s00246-009-9406-5. Epub 2009 Apr 25.

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 lead 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, a genetically heterogeneous disorder. Here we summarize our recent findings through the analyses of several genetically engineered mouse models that have defects in cardiac trabeculation and compaction. Our data indicate that cellular growth and differentiation signaling pathways are keys in these ventricular morphogenetic events.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Disease Models, Animal
  • Heart Ventricles / abnormalities*
  • Heart Ventricles / embryology*
  • Mice
  • Mice, Inbred Strains