Autor: |
Piotrowski, Tatjana, Dae-gwon Ahn, Schilling, Thomas F., Nair, Sreelaja, Ruvinsky, Ilya, Geisler, Robert, Rauch, Gerd-Jorg, Haffter, Pascal, Zon, Leonard I., Yi Zhou, Foott, Helen, Dawid, Igor B., Ho, Robert K. |
Předmět: |
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Zdroj: |
Development (09501991); Oct2003, Vol. 130 Issue 20, p5043, 10p, 8 Diagrams |
Abstrakt: |
The van gogh (vgo) mutant in zebrafish is characterized by defects in the ear, pharyngeal arches and associated structures such as the thymus. We show that vgo is caused by a mutation in tbx1, a member of the large family of T-box genes, tbx1 has been recently suggested to be a major contributor to the cardiovascular defects in DiGeorge deletion syndrome (DGS) in humans, a syndrome in which several neural crest derivatives are affected in the pharyngeal arches. Using cell transplantation studies, we demonstrate that vgo/tbx1 acts cell autonomously in the pharyngeal mesendoderm and influences the development of neural crest-derived cartilages secondarily. Furthermore, we provide evidence for regulatory interactions between vgo/tbx1 and edn1 and hand2, genes that are implicated in the control of pharyngeal arch development and in the etiology of DGS. [ABSTRACT FROM AUTHOR] |
Databáze: |
Complementary Index |
Externí odkaz: |
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