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J. Dev. Biol. 2017, 5(4), 17; doi:10.3390/jdb5040017

The Role of Hedgehog Signalling in the Formation of the Ventricular Septum

Institute for Animal Developmental and Molecular Biology, Heinrich-Heine University Düsseldorf, 40225 Düsseldorf, Germany
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Received: 30 November 2017 / Revised: 8 December 2017 / Accepted: 9 December 2017 / Published: 12 December 2017
(This article belongs to the Special Issue Hedgehog Signaling in Embryogenesis)
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Abstract

An incomplete septation of the ventricles in the vertebrate heart that disturbes the strict separation between the contents of the two ventricles is termed a ventricular septal defect (VSD). Together with bicuspid aortic valves, it is the most frequent congenital heart disease in humans. Until now, life-threatening VSDs are usually treated surgically. To avoid surgery and to develop an alternative therapy (e.g., a small molecule therapy), it is necessary to understand the molecular mechanisms underlying ventricular septum (VS) development. Consequently, various studies focus on the investigation of signalling pathways, which play essential roles in the formation of the VS. In the past decade, several reports found evidence for an involvement of Hedgehog (HH) signalling in VS development. In this review article, we will summarise the current knowledge about the association between HH signalling and VS formation and discuss the use of such knowledge to design treatment strategies against the development of VSDs. View Full-Text
Keywords: ventricular septal defect; VSD; Smoothened; SMO; SAG; purmorphamine; oxysterols; GSA-10; cilia; Down syndrome ventricular septal defect; VSD; Smoothened; SMO; SAG; purmorphamine; oxysterols; GSA-10; cilia; Down syndrome
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Wiegering, A.; Rüther, U.; Gerhardt, C. The Role of Hedgehog Signalling in the Formation of the Ventricular Septum. J. Dev. Biol. 2017, 5, 17.

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