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Aberrant processing forms of lung surfactant proteins SP-B and SP-C revealed by high-resolution mass spectrometry.

Abstract
The mutation (g.1286T>C) of the pulmonary surfactant-associated protein C gene (SFTPC) leads to the I73T substitution in the precursor protein (pro-SP-C) and results in interstitial lung disease with the histological pattern of non-specific interstitial pneumonia and pulmonary alveolar proteinosis. Central for the disease is the abnormal processing of the SP-C pro-protein to mature SP-C; however little is known about the nature of intermediates and processing products. We report here the application of high resolution Fourier transform ion cyclotron resonance (FT-ICR) mass spectrometry to the characterization of processing intermediates of hydrophobic pulmonary surfactant proteins SP-B and SP-C in intra- alveolar surfactant material of a patient with I73T mutation. SP-C and SP-B processing forms were separated from broncho-alveolar lavage fluid using chloroform/methanol extraction and sodium dodecyl sulfate poly acrylamide gel electrophoreis, detected by Western blot and identified by electrospray- and matrix-assisted laser desorption/ionization-FT-ICR mass spectrometry. The mass spectrometric and immuno-analytical results show the intra-alveolar accumulation of an aberrant C-terminal SP-C processing products in which the mature SP-C protein part is missing and aberrant processing intermediates of SP-B.
AuthorsDmitry Galetskiy, Markus Woischnik, Jan Ripper, Matthias Griese, Michael Przybylski
JournalEuropean journal of mass spectrometry (Chichester, England) (Eur J Mass Spectrom (Chichester)) Vol. 14 Issue 6 Pg. 379-90 ( 2008) ISSN: 1469-0667 [Print] England
PMID19136726 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Pulmonary Surfactant-Associated Protein B
  • Pulmonary Surfactant-Associated Protein C
  • SFTPC protein, human
Topics
  • Amino Acid Sequence
  • Bronchoalveolar Lavage Fluid (chemistry)
  • Fourier Analysis
  • Humans
  • Mass Spectrometry (methods)
  • Molecular Sequence Data
  • Pulmonary Surfactant-Associated Protein B (chemistry, genetics)
  • Pulmonary Surfactant-Associated Protein C (chemistry, genetics)
  • Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization

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