In vitro surfactant protein B deficiency inhibits lamellar body formation

Am J Respir Cell Mol Biol. 2003 Aug;29(2):259-66. doi: 10.1165/rcmb.2002-0149OC. Epub 2003 Mar 20.

Abstract

Surfactant protein (SP) B is essential for normal pulmonary surfactant activity and lamellar body genesis in type 2 cells. However, the role of SP-B in lamellar body genesis is poorly understood. We developed an adenovirus vector expressing antisense SP-B as an alternative in vitro model of SP-B deficiency to begin to explore the role of SP-B in lamellar body genesis. RT-PCR analysis revealed that antisense SP-B expression interfered with translation of endogenous SP-B mRNA. Antisense SP-B expression resulted in reliable in vitro reproduction of many features of SP-B deficiency, including absent mature SP-B and decreased lamellar bodies and SP-C. Light and electron microscopy demonstrated significant reductions in lamellar body number. Western blotting revealed a significant reduction in mature 8-kD SP-B protein and decreased mature SP-C. Our data indicate that antisense SP-B can be effectively used to replicate the SP-B-deficient type 2 cell phenotype in vitro, and provides an attractive alternative to transgenic models for the further study of the role of SP-B in lamellar body genesis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adenoviridae / genetics
  • Blotting, Western
  • Cell Line
  • Cell Survival
  • Cells, Cultured
  • Dose-Response Relationship, Drug
  • Genetic Vectors
  • Humans
  • Lung / metabolism
  • Lung / physiology*
  • Microscopy, Electron
  • Models, Genetic
  • Oligonucleotides, Antisense / pharmacology
  • Phenotype
  • Plasmids / metabolism
  • Pulmonary Surfactant-Associated Protein B / deficiency*
  • Pulmonary Surfactant-Associated Protein C / chemistry
  • RNA / metabolism
  • RNA, Messenger / metabolism
  • Reverse Transcriptase Polymerase Chain Reaction
  • Time Factors

Substances

  • Oligonucleotides, Antisense
  • Pulmonary Surfactant-Associated Protein B
  • Pulmonary Surfactant-Associated Protein C
  • RNA, Messenger
  • RNA