title: Surfactant-associated proteins: structure, function and clinical implications.
abstract: Surfactant replacement therapy is now the standard of care for infants with respiratory distress syndrome. As the understanding of surfactant structure and function has evolved, surfactant-associated proteins are now understood to be essential components of pulmonary surfactant. Their structural and functional diversity detail the complexity of their contributions to normal pulmonary physiology, and deficiency states result in significant pathology. Engineering synthetic surfactant protein constructs has been a major research focus for replacement therapies. This review highlights what is known about surfactant proteins and how this knowledge is pivotal for future advancements in treating respiratory distress syndrome as well as other pulmonary diseases characterized by surfactant deficiency or inactivation.
authors.forename: Anastasia K
authors.initials: AK
authors.lastname: Ketko
authors.affiliation: 8-621 C.S. Mott Children's Hospital, 1540 E. Hospital Drive, Ann Arbor, MI 48109-5254, USA.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Donn
mesh_terms: D001688:Biological Products / Q000378:metabolism* / Q000627:therapeutic use; D020022:Genetic Predisposition to Disease; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D008168:Lung / Q000378:metabolism* / Q000503:physiopathology; D037662:Pulmonary Surfactant-Associated Protein A / Q000172:deficiency; D037701:Pulmonary Surfactant-Associated Protein B / Q000172:deficiency; D037721:Pulmonary Surfactant-Associated Protein C / Q000172:deficiency; D037663:Pulmonary Surfactant-Associated Protein D / Q000172:deficiency; D037661:Pulmonary Surfactant-Associated Proteins / Q000378:metabolism* / Q000627:therapeutic use; D011663:Pulmonary Surfactants / Q000378:metabolism* / Q000627:therapeutic use; D012127:Respiratory Distress Syndrome, Newborn / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology
chemical_list: D001688:Biological Products; D037662:Pulmonary Surfactant-Associated Protein A; D037701:Pulmonary Surfactant-Associated Protein B; D037721:Pulmonary Surfactant-Associated Protein C; D037663:Pulmonary Surfactant-Associated Protein D; D037661:Pulmonary Surfactant-Associated Proteins; D011663:Pulmonary Surfactants