Article
Mouse lacking COUP-TFII as an animal model of Bochdalek-type congenital diaphragmatic hernia.
Proceedings of the National Academy of Sciences of the United States of America - 8 Nov 2005
You Li-Ru, Takamoto Norio, Yu Cheng-Tai, Tanaka Toshiya, Kodama Tatsuhiko, Demayo Francesco J, Tsai Sophia Y, Tsai Ming-Jer
Abstract excerpt
Congenital diaphragmatic hernia (CDH), a life-threatening anomaly, is a major cause of pediatric mortality. Although the disease was described >350 years ago, the etiology of CDH is poorly understood. Here, we show that tissue-specific null mutants of COUP-TFII exhibit Bochdalek-type CDH, the most common form of CDH. COUP-TFII, a member of orphan nuclear receptors, is expressed in regions critical for the...
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