Function of Surfactant Proteins B and C

Annu Rev Physiol. 2001;63:555-78. doi: 10.1146/annurev.physiol.63.1.555.

Abstract

SP-B is the only surfactant-associated protein absolutely required for postnatal lung function and survival. Complete deficiency of SP-B in mice and humans results in lethal, neonatal respiratory distress syndrome and is characterized by a virtual absence of lung compliance, highly disorganized lamellar bodies, and greatly diminished levels of SP-C mature peptide; in contrast, lung structure and function in SP-C null mice is normal. This review attempts to integrate recent findings in humans and transgenic mice with the results of in vitro studies to provide a better understanding of the functions of SP-B and SP-C and the structural basis for their actions.

Publication types

  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Humans
  • Molecular Sequence Data
  • Proteolipids / genetics*
  • Proteolipids / metabolism*
  • Pulmonary Alveoli / physiology*
  • Pulmonary Surfactants / genetics*
  • Pulmonary Surfactants / metabolism*

Substances

  • Proteolipids
  • Pulmonary Surfactants