Journal of Molecular Medicine

, Volume 75, Issue 8, pp 551–566

Animal models of congenital defects in the ventriculoarterial connection of the heart

Authors

  • Jing Ya
    • Department of Anatomy and Embryology, Academic Medical Center, University of Amsterdam, Meibergdreef 15, NL-1105 AZ, Amsterdam, The Netherlands
  • M. W. Schilham
    • Department of Immunology, University of Utrecht, The Netherlands
  • Hans Clevers
    • Department of Immunology, University of Utrecht, The Netherlands
  • Antoon F. M. Moorman
    • Department of Anatomy and Embryology, Academic Medical Center, University of Amsterdam, Meibergdreef 15, NL-1105 AZ, Amsterdam, The Netherlands
  • W. H. Lamers
    • Department of Anatomy and Embryology, Academic Medical Center, University of Amsterdam, Meibergdreef 15, NL-1105 AZ, Amsterdam, The Netherlands
REVIEW

DOI: 10.1007/s001090050140

Cite this article as:
Ya, J., Schilham, M., Clevers, H. et al. J Mol Med (1997) 75: 551. doi:10.1007/s001090050140

Abstract

 The embryonic heart functions as a pump without one-way valves. To accomplish this, a long, slowly conducting myocardial structure, the outflow tract, functions as a sphincter at the arterial pole of the heart. During subsequent development tissue remodeling in the outflow tract and immigrating cells of the neural crest are responsible for connecting the right ventricle with the pulmonary trunk and the left ventricle with the aorta, that is, for the developmental formation of the ventriculoarterial junction. Most congenital malformations of the ventriculoarterial junction stem from disturbances that result in developmental arrest or in abnormal pattern formation (”real” teratology). Abnormal pattern formation can in turn originate from problems with laterality or from aberrant or incomplete formation of structural elements. Genetically modified animals with well-defined gene deficiencies are beginning to provide insight in the signal-transduction pathways and structural elements that are responsible for normal development.

Key words HeartEmbryologyMalformationsAnimal modelsGenetic manipulation

Copyright information

© Springer-Verlag Berlin Heidelberg 1997