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Cited by 1,138

The DOI matches its Crossref record; 9 of the 9 references it cites were found in a registry or an index.

A novel source of arterial valve cells linked to bicuspid aortic valve without raphe in mice

Open access
Eley, Lorriane, Alqahtani, Ahlam MS, MacGrogan, Donal, Richardson, Rachel V, Murphy, Lindsay, Salguero-Jimenez, Alejandro, Sintes Rodriguez San Pedro, Marcos, Tiurma, Shindi, McCutcheon, Lauren, Gilmore, Adam, de La Pompa, José Luis, Chaudhry, Bill, Henderson, Deborah J·2018·eLife

Abstract

Abnormalities of the arterial valve leaflets, predominantly bicuspid aortic valve, are the commonest congenital malformations. Although many studies have investigated the development of the arterial valves, it has been assumed that, as with the atrioventricular valves, endocardial to mesenchymal transition (EndMT) is the predominant mechanism. We show that arterial is distinctly different from atrioventricular valve formation. Whilst the four septal valve leaflets are dominated by NCC and EndMT-derived cells, the intercalated leaflets differentiate directly from Tnnt2-Cre+/Isl1+ progenitors in the outflow wall, via a Notch-Jag dependent mechanism. Further, when this novel group of progenitors are disrupted, development of the intercalated leaflets is disrupted, resulting in leaflet dysplasia and bicuspid valves without raphe, most commonly affecting the aortic valve. This study thus overturns the dogma that heart valves are formed principally by EndMT, identifies a new source of valve interstitial cells, and provides a novel mechanism for causation of bicuspid aortic valves without raphe.

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Registry

Crossref · 10.7554/elife.34110

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9 of 9 references verified0 citation errors · 0 not checkable

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Metadata

Authors
Eley, L., Alqahtani, A. M., MacGrogan, D., Richardson, R. V., Murphy, L., Salguero-Jimenez, A., Sintes Rodriguez San Pedro, M., Tiurma, S., McCutcheon, L., Gilmore, A., de La Pompa, J. L., Chaudhry, B., & Henderson, D. J.
Venue
eLife
Published
2018-06-29
Volume
7
ISSN
2050-084X (ISSN-L)
OpenAlex
W2810440784
Cited by
1,138
References
9
FWCI
43.08
Type
journal article
Language
en
Open access
gold

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