期刊
DEVELOPMENT
卷 145, 期 1, 页码 -出版社
COMPANY BIOLOGISTS LTD
DOI: 10.1242/dev.151944
关键词
Neural crest; Cardiac development; Krox20; Mouse; Genetics; Bicuspid aortic valve; Egr2
资金
- Association Francaise contre les Myopathies (AFM-Telethon) (NMH-Decrypt Project)
- Association Francaise contre les Myopathies (AFM-Telethon) (TRIM-RD Project)
- Fondation pour la Recherche Medicale (FRM) [DPC20111123002]
- Fondation coeur et arteres
- Association pour la Recherche en Cardiologie du Foetus a l'Adulte (ARCFA)
- Institut National de la Sante et de la Recherche Medicale (INSERM)
- Fondation pour la Recherche Medicale (FRM)
- Institut National de la Sante et de la Recherche Medicale
- Fondation Lefoulon Delalande
- Association Francais contre les Myopathies
Although cardiac neural crest cells are required at early stages of arterial valve development, their contribution during valvular leaflet maturation remains poorly understood. Here, we show in mouse that neural crest cells from pre-otic and post-otic regions make distinct contributions to the arterial valve leaflets. Genetic fate-mapping analysis of Krox20-expressing neural crest cells shows a large contribution to the borders and the interleaflet triangles of the arterial valves. Loss of Krox20 function results in hyperplastic aortic valve and partially penetrant bicuspid aortic valve formation. Similar defects are observed in neural crest Krox20-deficient embryos. Genetic lineage tracing in Krox20(-/-) mutant mice shows that endothelial-derived cells are normal, whereas neural crest-derived cells are abnormally increased in number and misplaced in the valve leaflets. In contrast, genetic ablation of Krox20-expressing cells is not sufficient to cause an aortic valve defect, suggesting that adjacent cells can compensate this depletion. Our findings demonstrate a crucial role for Krox20 in arterial valve development and reveal that an excess of neural crest cells may be associated with bicuspid aortic valve.
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