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

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

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GALETSKIY, Dmitry, Markus WOISCHNIK, Jan RIPPER, Matthias GRIESE, Michael PRZYBYLSKI, 2008. Aberrant processing forms of lung surfactant proteins SP B and SP C revealed by high-resolution mass spectrometry. In: European Journal of Mass Spectrometry. 14(6), pp. 379-390. ISSN 1356-1049. Available under: doi: 10.1255/ejms.957

@article{Galetskiy2008Aberr-1059, title={Aberrant processing forms of lung surfactant proteins SP B and SP C revealed by high-resolution mass spectrometry}, year={2008}, doi={10.1255/ejms.957}, number={6}, volume={14}, issn={1356-1049}, journal={European Journal of Mass Spectrometry}, pages={379--390}, author={Galetskiy, Dmitry and Woischnik, Markus and Ripper, Jan and Griese, Matthias and Przybylski, Michael} }

2008 Galetskiy, Dmitry 2011-03-22T17:55:01Z First publ. in: European Journal of Mass Spectrometry 14 (2008), 6, pp. 379-390 Woischnik, Markus 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. Griese, Matthias Aberrant processing forms of lung surfactant proteins SP B and SP C revealed by high-resolution mass spectrometry eng Woischnik, Markus Przybylski, Michael 2011-03-22T17:55:01Z Galetskiy, Dmitry Przybylski, Michael Griese, Matthias Ripper, Jan Ripper, Jan deposit-license

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