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The Role of Cerl2 in the Establishment of Left-Right Asymmetries during Axis Formation and Heart Development

The formation of the asymmetric left-right (LR) body axis is one of the fundamental aspects of vertebrate embryonic development, and one still raising passionate discussions among scientists. Although the conserved role of nodal is unquestionable in this process, several of the details around this s...

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Autores principales: Belo, José A., Marques, Sara, Inácio, José M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5753124/
https://www.ncbi.nlm.nih.gov/pubmed/29367552
http://dx.doi.org/10.3390/jcdd4040023
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author Belo, José A.
Marques, Sara
Inácio, José M.
author_facet Belo, José A.
Marques, Sara
Inácio, José M.
author_sort Belo, José A.
collection PubMed
description The formation of the asymmetric left-right (LR) body axis is one of the fundamental aspects of vertebrate embryonic development, and one still raising passionate discussions among scientists. Although the conserved role of nodal is unquestionable in this process, several of the details around this signaling cascade are still unanswered. To further understand this mechanism, we have been studying Cerberus-like 2 (Cerl2), an inhibitor of Nodal, and its role in the generation of asymmetries in the early vertebrate embryo. The absence of Cerl2 results in a wide spectrum of malformations commonly known as heterotaxia, which comprises defects in either global organ position (e.g., situs inversus totalis), reversed orientation of at least one organ (e.g., situs ambiguus), and mirror images of usually asymmetric paired organs (e.g., left or right isomerisms of the lungs). Moreover, these laterality defects are frequently associated with congenital heart diseases (e.g., transposition of the great arteries, or atrioventricular septal defects). Here, reviewing the knowledge on the establishment of LR asymmetry in mouse embryos, the emerging conclusion is that as necessary as is the activation of the Nodal signaling cascade, the tight control that Cerl2-mediates on Nodal signaling is equally important, and that generates a further regionalized LR genetic program in the proper time and space.
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spelling pubmed-57531242018-01-19 The Role of Cerl2 in the Establishment of Left-Right Asymmetries during Axis Formation and Heart Development Belo, José A. Marques, Sara Inácio, José M. J Cardiovasc Dev Dis Review The formation of the asymmetric left-right (LR) body axis is one of the fundamental aspects of vertebrate embryonic development, and one still raising passionate discussions among scientists. Although the conserved role of nodal is unquestionable in this process, several of the details around this signaling cascade are still unanswered. To further understand this mechanism, we have been studying Cerberus-like 2 (Cerl2), an inhibitor of Nodal, and its role in the generation of asymmetries in the early vertebrate embryo. The absence of Cerl2 results in a wide spectrum of malformations commonly known as heterotaxia, which comprises defects in either global organ position (e.g., situs inversus totalis), reversed orientation of at least one organ (e.g., situs ambiguus), and mirror images of usually asymmetric paired organs (e.g., left or right isomerisms of the lungs). Moreover, these laterality defects are frequently associated with congenital heart diseases (e.g., transposition of the great arteries, or atrioventricular septal defects). Here, reviewing the knowledge on the establishment of LR asymmetry in mouse embryos, the emerging conclusion is that as necessary as is the activation of the Nodal signaling cascade, the tight control that Cerl2-mediates on Nodal signaling is equally important, and that generates a further regionalized LR genetic program in the proper time and space. MDPI 2017-12-10 /pmc/articles/PMC5753124/ /pubmed/29367552 http://dx.doi.org/10.3390/jcdd4040023 Text en © 2017 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Belo, José A.
Marques, Sara
Inácio, José M.
The Role of Cerl2 in the Establishment of Left-Right Asymmetries during Axis Formation and Heart Development
title The Role of Cerl2 in the Establishment of Left-Right Asymmetries during Axis Formation and Heart Development
title_full The Role of Cerl2 in the Establishment of Left-Right Asymmetries during Axis Formation and Heart Development
title_fullStr The Role of Cerl2 in the Establishment of Left-Right Asymmetries during Axis Formation and Heart Development
title_full_unstemmed The Role of Cerl2 in the Establishment of Left-Right Asymmetries during Axis Formation and Heart Development
title_short The Role of Cerl2 in the Establishment of Left-Right Asymmetries during Axis Formation and Heart Development
title_sort role of cerl2 in the establishment of left-right asymmetries during axis formation and heart development
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5753124/
https://www.ncbi.nlm.nih.gov/pubmed/29367552
http://dx.doi.org/10.3390/jcdd4040023
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