Aberrant processing of surfactant protein C in hereditary SP-B deficiency

D. K. Vorbroker, S. A. Profitt, L. M. Nogee, J. A. Whitsett

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181 Scopus citations


Hereditary surfactant protein B (SP-B) deficiency causes lethal neonatal respiratory disease associated with abnormalities in pulmonary surfactant proteins and lipids. SP-C, a 4-kDa hydrophobic protein produced from a 21- kDa precursor, cooperates with SP-B to enhance the surface active properties of surfactant phospholipids. Anti-proSP-C polyclonal antisera were produced against fusion proteins containing 1) the amino terminus (amino acids 1-20), 2) the region carboxy-terminal to the mature SP-C peptide (amino acids 58- 77), and 3) full-length 197-amino acid proSP-C and were characterized using immunoprecipitation, Western blot, and immunohistochemical techniques. Western blot analysis of bronchoalveolar lavage and amniotic fluid from hereditary SP-B-deficient patients glowed identification of a 12-kDa form of SP-C that contained epitopes consistent with the amino-terminal and active peptide regions of SP-C (amino acids 1-57). The 12-kDa SP-C peptide was not detected in bronchoalveolar lavage from healthy adults or adults with alveolar proteinosis or pneumonia. We conclude that SP-B deficiency is associated with the aberrant processing and secretion of an immature SP-C peptide, which may contribute to the respiratory failure associated with hereditary SP-B deficiency.

Original languageEnglish (US)
Pages (from-to)L647-L656
JournalAmerican Journal of Physiology - Lung Cellular and Molecular Physiology
Issue number4 12-4
StatePublished - 1995
Externally publishedYes


  • protein processing
  • regulated secretion
  • type II cells

ASJC Scopus subject areas

  • Physiology
  • Pulmonary and Respiratory Medicine
  • Physiology (medical)
  • Cell Biology


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