[Cilia and heart morphogenesis].

Med Sci (Paris)

Institut Pasteur, Département de biologie du développement et cellules souches, 25, rue du Docteur Roux, 75015 Paris, France - CNRS URA2578, 75015 Paris, France.

Published: November 2014

AI Article Synopsis

  • - The discovery in 2000 identified primary cilia as crucial organelles for embryonic development, leading to the creation of mouse models to study ciliopathies, which often result in heart malformations.
  • - Primary cilia are essential for determining left/right orientation in embryos, significantly affecting heart formation, with abnormal heart looping being a common issue in ciliopathy models.
  • - The role of primary cilia in cardiac cells is not well understood, but signaling pathways involving receptors located in cilia may influence heart growth and development, highlighting the need for more research to understand their contribution to heart-related diseases.

Article Abstract

After the seminal discovery in 2000 that primary cilia are functional organelles which are essential for embryonic development, several mouse models of ciliopathies have been generated. The heart is frequently affected, with a large spectrum of malformations. The cilia of the node are required early in development in the determination of the left/right laterality of the embryo, which has secondary consequences on the formation of the heart. Thus, abnormal looping of the heart is a recurrent phenotype in models of ciliopathies. However, the function of primary cilia in cardiac cells remains poorly understood. Receptors such as polycystins or hedgehog receptors are usually localized in the primary cilium, raising the possibility that these signalling pathways, which are important for the septation and the growth of the heart, are transduced in primary cilia of cardiac cells. Knowledge of the roles of primary cilia at different steps of heart development and in different cardiac cell types will be essential to better understand the origin of human cardiopathies associated with ciliopathies.

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Source
http://dx.doi.org/10.1051/medsci/20143011014DOI Listing

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