title: Molecular mechanism of ventricular trabeculation/compaction and the pathogenesis of the left ventricular noncompaction cardiomyopathy (LVNC).
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.
authors.affiliation: Riley Heart Research Center, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
authors.forename: Wenjun
authors.initials: W
authors.lastname: Zhang
authors.forename: Hanying
authors.initials: H
authors.lastname: Chen
authors.forename: Xiuxia
authors.initials: X
authors.lastname: Qu
authors.forename: Ching-Pin
authors.initials: CP
authors.lastname: Chang
authors.forename: Weinian
authors.initials: W
authors.lastname: Shou
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D020022:Genetic Predisposition to Disease; D006352:Heart Ventricles / Q000002:abnormalities* / Q000196:embryology; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000235:genetics* / Q000503:physiopathology; D051379:Mice; D008822:Mice, Transgenic; D009154:Mutation; D022061:Tacrolimus Binding Protein 1A / Q000235:genetics / Q000378:metabolism
chemical_list: D022061:Tacrolimus Binding Protein 1A