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.
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Authors | Dmitry Galetskiy, Markus Woischnik, Jan Ripper, Matthias Griese, Michael Przybylski |
Journal | European journal of mass spectrometry (Chichester, England)
(Eur J Mass Spectrom (Chichester))
Vol. 14
Issue 6
Pg. 379-90
( 2008)
ISSN: 1469-0667 [Print] England |
PMID | 19136726
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Pulmonary Surfactant-Associated Protein B
- Pulmonary Surfactant-Associated Protein C
- SFTPC protein, human
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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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