Function of surfactant proteins B and C.
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.
Weaver
T E
TE
Division of Pulmonary Biology, Children's Hospital Medical Center, Cincinnati, Ohio 45229-3039, USA. tim.weaver@chmcc.org
Conkright
J J
JJ
D000595:Amino Acid Sequence; D000818:Animals; D006801:Humans; D008969:Molecular Sequence Data; D011510:Proteolipids / Q000235:genetics* / Q000378:metabolism*; D011650:Pulmonary Alveoli / Q000502:physiology*; D011663:Pulmonary Surfactants / Q000235:genetics* / Q000378:metabolism*
D011510:Proteolipids; D011663:Pulmonary Surfactants