Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Oct 21;147(21):dev194233.
doi: 10.1242/dev.194233.

The developing heart: from The Wizard of Oz to congenital heart disease

Benoit G Bruneau  1   2   3   4
Affiliations
Review

The developing heart: from The Wizard of Oz to congenital heart disease

Benoit G Bruneau. Development. .

Abstract

The heart is an essential organ with a fascinating developmental biology. It is also one of the organs that is most often affected in human disease, either during development or in postnatal life. Over the last few decades, insights into the development of the heart have led to fundamental new concepts in gene regulation, but also to genetic and mechanistic insights into congenital heart defects. In more recent years, the lessons learned from studying heart development have been applied to interrogating regeneration of the diseased heart, exemplifying the importance of understanding the mechanistic underpinnings that lead to the development of an organ.

Keywords: Congenital heart disease; Gene regulation; Heart development; Regeneration.

PubMed Disclaimer

Conflict of interest statement

Competing interestsB.G.B. is a co-founder of Tenaya Therapeutics, which aims to use developmentally important factors as therapies for heart failure.

Figures

Fig. 1.
Fig. 1.
The tinman Drosophila phenotype. (A) A wild-type Drosophila embryo stained for eve, which marks neural cell (nc) precursors, anal plate (ap) precursors and pericardial cell (pc) precursors. (B) Staining for eve in a tinman mutant embryo reveals an absence of pericardial cell precursors. This figure was reproduced unmodified with permission from Azpiazu and Frasch (1993), where it was published under a CC-BY-NC 4.0 licence.
Fig. 2.
Fig. 2.
Conserved expression of tinman and its vertebrate homologs. (A-H) The expression of tinman and its vertebrate homologs in heart progenitor cells is shown in Drosophila (A), Xenopus (B, stage 20 embryo), mouse (E.7.5, C; E8.75, D), chick (stage 6, E; stage 10, F) and zebrafish (19.5 h, G; 24 h, H) embryos. Reproduced with permission from Harvey (1996).
Fig. 3.
Fig. 3.
Transcriptional network underlying heart development. The gene regulatory network underlying heart development is shown. Genes that have been implicated in human CHD are highlighted (red ovals). Adapted from Davidson (2010).
See this image and copyright information in PMC

References

    1. Azpiazu N. and Frasch M. (1993). tinman and bagpipe: two homeo box genes that determine cell fates in the dorsal mesoderm of Drosophila. Genes Dev. 7, 1325-1340. 10.1101/gad.7.7b.1325 - DOI - PubMed
    1. Basson C. T., Bachinsky D. R., Lin R. C., Levi T., Elkins J. A., Soults J., Grayzel D., Kroumpouzou E., Traill T. A., Leblanc-Straceski J. et al. (1997). Mutations in human cause limb and cardiac malformation in Holt-Oram syndrome. Nat. Genet. 15, 30-35. 10.1038/ng0197-30 - DOI - PubMed
    1. Benson D. W., Silberbach G. M., Kavanaugh-McHugh A., Cottrill C., Zhang Y., Riggs S., Smalls O., Johnson M. C., Watson M. S., Seidman J. G. et al. (1999). Mutations in the cardiac transcription factor NKX2.5 affect diverse cardiac developmental pathways. J. Clin. Invest. 104, 1567-1573. 10.1172/JCI8154 - DOI - PMC - PubMed
    1. Bodmer R. (1993). The gene tinman is required for specification of the heart and visceral muscles in Drosophila. Development 118, 719-729. - PubMed
    1. Bodmer R. (1995). Heart development in Drosophila and its relationship to vertebrates. Trends Cardiovasc. Med. 5, 21-28. 10.1016/1050-1738(94)00032-Q - DOI - PubMed

Publication types