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

Authors

  • Wenjun Zhang,

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    • Wenjun Zhang, M.D. is a research assistant professor of Pediatrics, Riley Heart Research Center, Indiana University School of Medicine. His research focuses on molecular signaling in regulating mammalian heart development and heart failure.
  • Hanying Chen,

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    • Hanying Chen, M.D., is a research assistant professor of Pediatrics, Riley Heart Research Center, Indiana University School of Medicine. His research focuses on TGFβ/BMP signaling in regulating ventricular wall development and maturation.
  • Xiuxia Qu,

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    • Xiuxia Qu, Ph.D., is a research associate at Department of Medical and Molecular Genetics, Indiana University School of Medicine. Her research interests are FGF signaling and morphogens in mammalian embryonic development and organogenesis.
  • Ching-Pin Chang,

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    • Ching-Pin Chang, M.D., Ph.D. is Associate Professor of Medicine, Division of Cardiovascular Medicine, Division of Cardiology, Department of Medicine, Indiana University School of Medicine; Department of Medicine, Stanford University School of Medicine. His research focuses on the epigenetic regulation of cardiovascular development and heart failure, and the translation of the bench findings to clinical applications.
  • Weinian Shou

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    • Weinian Shou, Ph.D. is Professor of Pediatrics, Biochemistry & Molecular Biology, Medical & Molecular Genetics, and Pharmacology & Toxicology, Indiana University School of Medicine. His research focuses on the molecular signaling regulating cardiac development and the pathogenesis of congenital heart defects, with particular emphasis on ventricle wall formation and maturation.

  • Conflict of interest: none.

Correspondence to: Dr. Weinian Shou, Ph.D., Riley Heart Research Center, Herman B. Wells Center for Pediatric Research, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN 46202. E-mail: wshou@iu.edu

Correspondence to: Dr. Ching-Pin Chang, M.D., Ph.D., Krannert Institute of Cardiology and Division of Cardiology, Department of Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA; Division of Cardiovascular Medicine, Department of Medicine, Stanford University, School of Medicine, Stanford, CA 94305. E-mail: chingpin@standford.edu; bioxchang@gmail.com

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.

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