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Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice.

Surfactant protein B (SP-B) is an 8.7-kDa, hydrophobic protein that enhances the spreading and stability of surfactant phospholipids in the alveolus. To further assess the role of SP-B in lung function, the SP-B gene was disrupted by homologous recombination in murine mouse embryonic stem cells. Mice with a single mutated SP-B allele (+/-) were unaffected, whereas homozygous SP-B -/- offspring died of respiratory failure immediately after birth. Lungs of SP-B -/- mice developed normally but remained atelectatic in spite of postnatal respiratory efforts. SP-B protein and mRNA were undetectable and tubular myelin figures were lacking in SP-B -/- mice. Type II cells of SP-B -/- mice contained no fully formed lamellar bodies. While the abundance of SP-A and SP-C mRNAs was not altered, an aberrant form of pro-SP-C, 8.5 kDa, was detected, and fully processed SP-C peptide was markedly decreased in lung homogenates of SP-B -/- mice. Ablation of the SP-B gene disrupts the routing, storage, and function of surfactant phospholipids and proteins, causing respiratory failure at birth.

Pubmed ID: 7644495

Authors

  • Clark JC
  • Wert SE
  • Bachurski CJ
  • Stahlman MT
  • Stripp BR
  • Weaver TE
  • Whitsett JA

Journal

Proceedings of the National Academy of Sciences of the United States of America

Publication Data

August 15, 1995

Associated Grants

  • Agency: NHLBI NIH HHS, Id: HL38859
  • Agency: NHLBI NIH HHS, Id: HL41496
  • Agency: NHLBI NIH HHS, Id: HL51835

Mesh Terms

  • Animals
  • Animals, Newborn
  • Embryo, Mammalian
  • Epithelium
  • Genetic Vectors
  • Genomic Library
  • Homeostasis
  • Homozygote
  • Humans
  • Lung
  • Mice
  • Mice, Transgenic
  • Microscopy, Electron
  • Mutagenesis
  • Proteolipids
  • Pulmonary Alveoli
  • Pulmonary Surfactants
  • RNA, Messenger
  • Recombinant Proteins
  • Reference Values
  • Respiratory Insufficiency
  • Restriction Mapping
  • Stem Cells