Boucherat Olivier, Jeannotte Lucie, Hadchouel Alice, Delacourt Christophe, Benachi Alexandra
Institut Universitaire de Cardiologie et de Pneumologie de Québec, Université Laval, Québec, QC, Canada, G1 V 4G5.
Centre de recherche sur le cancer de l'Université Laval, CRCHUQ, L'Hôtel-Dieu de Québec, QC, Canada; Département de Biologie Moléculaire, Biochimie Médicale et Pathologie, Université Laval, Québec, Canada.
Paediatr Respir Rev. 2016 Jun;19:62-8. doi: 10.1016/j.prrv.2015.11.011. Epub 2016 Feb 3.
It is well established that a number of birth defects are associated with improper formation of the respiratory tract. Important progress has been made in the identification of components of the regulatory networks controlling lung morphogenesis. They comprise a variety of soluble factors, receptors, transcription factors, and miRNAs. However, the underlying molecular mechanisms remain unsolved and fundamental questions, such as those related to lung branching are still unanswered. Congenital cystic lung diseases consist of a heterogeneous group of rare lung diseases mainly detected prenatally and characterized by airway dilatation. Despite their apparent phenotypic heterogeneity, these malformations are proposed to be related to a common malformation sequence occurring during lung branching morphogenesis.
众所周知，许多出生缺陷与呼吸道的异常形成有关。在识别控制肺形态发生的调控网络的组成部分方面已经取得了重要进展。它们包括多种可溶性因子、受体、转录因子和微小RNA。然而，潜在的分子机制仍未解决，一些基本问题，如与肺分支相关的问题，仍然没有答案。先天性囊性肺病是一组异质性的罕见肺病，主要在产前被检测到，其特征是气道扩张。尽管它们在表型上明显异质性，但这些畸形被认为与肺分支形态发生过程中出现的共同畸形序列有关。
