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• W2182145751 abstract "Mammalian pulmonary surfactants contain four distinct surfactant proteins, termed surfactant proteins A, B, C, and D (SP-A, SP-B, SP-C, and SP-D), that play unique roles in host defense, surfactant phospholipid homeostasis, and surfactant function. While in vitro studies supported overlapping roles for the surfactant proteins, unique functions for each surfactant protein were identified by studies in transgenic mice in which the surfactant proteins were deleted by gene targeting. Findings in transgenic mice lacking SP-A and SP-D demonstrated that each plays distinct roles in host defense, and in the regulation of surfactant structure and metabolism. Targeting of SP-B gene demonstrated its critical role in lung function after birth; mutations in the mouse or human SP-B gene caused respiratory failure in the immediate postnatal period. Likewise, mutations in the SP-C gene altered pulmonary function in mice. Thus, surfactant proteins play distinct and important roles in pulmonary function and host defense. The critical role of the reduction of surface tension at the air-liquid interface in the pathophysiology of respiratory distress syndrome and the transition to air breathing after birth was recognized more than 40 years ago. 1 Surfactant phospholipids play a critical role in reducing surface tension in the alveolus, and the lack of surfactant causes respiratory distress syndrome (RDS) in preterm infants. In subsequent years intense research activities were focused to determine the composition and function of pulmonary surfactants. 2 While consisting predominantly of phospholipids, mammalian surfactant also contain neutral lipids, fatty acids, and proteins. Four distinct surfactant proteins, now termed SP-A, SP-B, SP- C, and SP-D, were identified in pulmonary surfactants. In the human lung, surfactant proteins are synthesized and secreted by respiratory epithelial cells, including Type II epithelial cells in the alveolus, and subsets of non-ciliated secretory cells in tracheal glands and conducting airways. 3-6 The structures of surfactant proteins were further elucidated by cDNA cloning, nucleotide and polypeptide sequencing that provided their primary structures. More recently, the role of surfactant proteins in lung function was determined by gene targeting in transgenic mice." @default.
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• W2182145751 date "2001-01-01" @default.
• W2182145751 modified "2023-09-27" @default.
• W2182145751 title "Functions of Surfactant Proteins in Vivo Discerned by Gene Targeting in Transgenic Mice" @default.
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