Hydrophobic Surfactant Proteins in Lung Function and Disease

doi:10.1056/NEJMra022387

Volume 347:2141-2148		December 26, 2002		Number 26

Hydrophobic Surfactant Proteins in Lung Function and Disease

Jeffrey A. Whitsett, M.D., and Timothy E. Weaver, Ph.D.

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The hydrophobic surfactant proteins B and C are essential forlung function and pulmonary homeostasis after birth. These proteinsenhance the spreading, adsorption, and stability of surfactantlipids required for the reduction of surface tension in thealveolus. Surfactant proteins B and C also participate in theregulation of intracellular and extracellular processes criticalfor the maintenance of respiratory structure and function. Mutationsin the genes encoding surfactant protein B and surfactant proteinC (SFTPB and SFTPC, respectively) are associated with acuterespiratory failure and interstitial lung diseases. In thisarticle, we review the current knowledge regarding the structure. . . [Full Text of this Article]

Structure and Function of Alveolar Surfactant

Surfactant Protein B

Structure and Function

Critical Role in Perinatal Lung Function

Hereditary Surfactant Protein B Deficiency

Association of Chronic Lung Disease with Partial Surfactant Protein B Deficiency

Surfactant Protein C

Structure and Function

Role in Pulmonary Function and Homeostasis in Vivo

Interstitial Pneumonitis Caused by Mutations in SFTPC

Proposed Model for the Pathogenesis of Pulmonary Disease Caused by Mutations in SFTPB and SFTPC

Source Information

From the Divisions of Neonatology and Pulmonary Biology, Cincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati.

Address reprint requests to Dr. Whitsett at the Cincinnati Children's Hospital Medical Center, Divisions of Neonatology and Pulmonary Biology, 3333 Burnet Ave., Cincinnati, OH 45229-3039, or at jeff.whitsett@chmcc.org.

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