File:Nipbl heart and organ patterning.png

Multiple influences of Nipbls on regulatory networks underlying heart and visceral organ development

Nipped-B-like (Nipbl) - a highly conserved protein that facilitates cohesin loading.

Endoderm development is initiated by Nodal-dependent activation of a network of transcription factors. Within this network, Nipbls regulate expression of gata5, sox32, sox17, and foxa2. Sox32 is required for specification and maintenance of endoderm cell fate, and reduced levels of Sox32 in nipbla/b-morphants may reduce endodermal cell number. This, in turn, could produce in a thinner gut, and smaller visceral organs, as well as a failure of cardiac progenitors that migrate along endoderm to form a midline heart tube.

In parallel, a network triggered by cilia-driven fluid flow in KV underlies the establishment of L/R asymmetry in the embryo. Within this network, Nipbls regulate expression of dnah9, as well as the asymmetric expression of spaw and lefty2. Disruption in the expression of these genes is likely to underlie the heart jogging/looping, and gut looping defects in nipbla/b-morphants.

OMIM - CDLS1 | CDLS2 | CDLS3

Reference

Muto A, Calof AL, Lander AD & Schilling TF. (2011). Multifactorial origins of heart and gut defects in nipbl-deficient zebrafish, a model of Cornelia de Lange Syndrome. PLoS Biol. , 9, e1001181. PMID: 22039349 DOI.

Copyright

© 2011 Muto et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Figure 9. Journal.pbio.1001181.g009.png

Cite this page: Hill, M.A. (2024, April 19) Embryology Nipbl heart and organ patterning.png. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/File:Nipbl_heart_and_organ_patterning.png

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