期刊
PEDIATRIC SURGERY INTERNATIONAL
卷 36, 期 9, 页码 991-997出版社
SPRINGER
DOI: 10.1007/s00383-020-04705-0
关键词
Congenital diaphragmatic hernia; Pulmonary hypoplasia; Lung development; Pulmonary hypertension; Transgenic mice; Retinoic acid
资金
- Projekt DEAL
Congenital diaphragmatic hernia (CDH) is a relatively common and life-threatening birth defect, characterized by incomplete formation of the diaphragm. Because CDH herniation occurs at the same time as preacinar airway branching, normal lung development becomes severely disrupted, resulting almost invariably in pulmonary hypoplasia. Despite various research efforts over the past decades, the pathogenesis of CDH and associated lung hypoplasia remains poorly understood. With the advent of molecular techniques, transgenic animal models of CDH have generated a large number of candidate genes, thus providing a novel basis for future research and treatment. This review article offers a comprehensive overview of genes and signaling pathways implicated in CDH etiology, whilst also discussing strengths and limitations of transgenic animal models in relation to the human condition.
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