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

Wenjun Zhang, Hanying Chen, Xiuxia Qu, Ching Pin Chang, Weinian Shou

Research output: Contribution to journalArticle

65 Scopus citations

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. © 2013 Wiley Periodicals, Inc.

Original languageEnglish (US)
Pages (from-to)144-156
Number of pages13
JournalAmerican Journal of Medical Genetics, Part C: Seminars in Medical Genetics
Volume163
Issue number3
DOIs
StatePublished - Aug 1 2013

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

Fingerprint Dive into the research topics of 'Molecular mechanism of ventricular trabeculation/compaction and the pathogenesis of the left ventricular noncompaction cardiomyopathy (LVNC)'. Together they form a unique fingerprint.

  • Cite this