Regulation and function of pulmonary surfactant protein B.
Pulmonary surfactant, a complex mixture of phospholipids and specific associated proteins, reduces the surface tension at the air-liquid interface of the distal conducting airways and gas exchanging alveoli of the lung. Lipids, primarily neutral and phospholipids, compose approximately 90% of the surfactant complex. The remaining 10% of surfactant is composed of at least three surfactant-specific proteins, designated surfactant protein A (SP-A), SP-B, and SP-C. These proteins contribute to the formation, stabilization, and function of organized surfactant structures. This article briefly reviews the normal composition and function of pulmonary surfactant and specifically reviews the structure, function, and regulation of surfactant protein B (SP-B). The recent identification of neonates with refractory respiratory failure due to a genetic absence of SP-B and the study of transgenic mice in which SP-B gene expression has been ablated highlight the importance of the protein to surfactant function, synthesis, and metabolism and to the maintenance of lung function. Gene reconstitution experiments in vitro and in SP-B-deficient transgenic mice suggest specific functions for the amino and carboxyl terminal domains of the protein. SP-B deficiency is a potential target for gene therapy in human patients.
Pryhuber
G S
GS
Department of Pediatrics, Strong Children's Hospital, Rochester, New York 14642, USA.
D000595:Amino Acid Sequence; D000818:Animals; D005786:Gene Expression Regulation; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D008969:Molecular Sequence Data; D011499:Protein Processing, Post-Translational; D011510:Proteolipids / Q000096:biosynthesis / Q000235:genetics / Q000502:physiology*; D011663:Pulmonary Surfactants / Q000096:biosynthesis / Q000235:genetics / Q000502:physiology*; D013329:Structure-Activity Relationship
D011510:Proteolipids; D011663:Pulmonary Surfactants