先天性气管缺陷:胚胎发育和动物模型

Z. Sher, Karen J. Liu
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引用次数: 11

摘要

气管异常是一种潜在的灾难性先天性缺陷。当新生儿开始呼吸时,气管需要保持弹性和刚性的适当平衡。如果气管软骨组织紊乱或结构薄弱,气道会塌陷,阻碍呼吸。软骨环太小或太硬也会阻碍呼吸。这些异常通常与颅面综合征相关,尽管其重要性,但人们对其了解甚少。在这篇综述中,我们总结了气管的病理表型谱,并将它们与小鼠模型中发现的分子事件联系起来。
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Congenital tracheal defects: embryonic development and animal models
Abstract Tracheal anomalies are potentially catastrophic congenital defects. As a newborn begins to breathe, the trachea needs to maintain an appropriate balance of elasticity and rigidity. If the tracheal cartilages are disorganized or structurally weak, the airways can collapse, obstructing breathing. Cartilage rings that are too small or too rigid can also obstruct breathing. These anomalies are frequently associated with craniofacial syndromes, and, despite the importance, are poorly understood. In this review, we summarize the spectrum of pathological phenotypes of the trachea and correlate them with the molecular events uncovered in mouse models.
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AIMS Genetics
AIMS Genetics GENETICS & HEREDITY-
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