title: [Pulmonary surfactant homeostasis associated genetic abnormalities and lung diseases].
abstract: Pulmonary surfactant (PS) is synthesized and secreted by alveolar epithelial type II (AEII) cells, which is a complex compound formed by proteins and lipids. Surfactant participates in a range of physiological processes such as reducing the surface tension, keeping the balance of alveolar fluid, maintaining normal alveolar morphology and conducting host defense. Genetic disorders of the surfactant homeostasis genes may result in lack of surfactant or cytotoxicity, and lead to multiple lung diseases in neonates, children and adults, including neonatal respiratory distress syndrome, interstitial pneumonia, pulmonary alveolar proteinosis, and pulmonary fibrosis. This paper has provided a review for the functions and processes of pulmonary surfactant metabolism, as well as the connection between disorders of surfactant homeostasis genes and lung diseases.
authors.affiliation: Institute of Animal Sciences, Chinese Academy of Agricultural Science, Beijing 100193, China; College of Animal Science and Technology, Northwest Agricultural and Forestry University, Yangling, Shaanxi 712100, China. Email: liuyan05@caas.cn.
authors.forename: Xiaojing
authors.initials: X
authors.lastname: Jiang
authors.forename: Xiuzhu
authors.initials: X
authors.lastname: Sun
authors.forename: Weihua
authors.initials: W
authors.lastname: Du
authors.forename: Haisheng
authors.initials: H
authors.lastname: Hao
authors.forename: Xueming
authors.initials: X
authors.lastname: Zhao
authors.forename: Dong
authors.initials: D
authors.lastname: Wang
authors.forename: Huabin
authors.initials: H
authors.lastname: Zhu
authors.forename: Yan
authors.initials: Y
authors.lastname: Liu
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000235:genetics; D004268:DNA-Binding Proteins / Q000235:genetics; D006706:Homeostasis; D006801:Humans; D008171:Lung Diseases / Q000235:genetics*; D037721:Pulmonary Surfactant-Associated Protein C / Q000235:genetics; D011663:Pulmonary Surfactants / Q000378:metabolism*; D014157:Transcription Factors
chemical_list: C441524:ABCA3 protein, human; D018528:ATP-Binding Cassette Transporters; D004268:DNA-Binding Proteins; D037721:Pulmonary Surfactant-Associated Protein C; D011663:Pulmonary Surfactants; C489679:SFTPC protein, human; C504003:TTF1 protein, human; D014157:Transcription Factors