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Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease
BACKGROUND: Abnormalities of the intracellular metabolism of the hydrophobic surfactant proteins SP-B and SP-C and their precursors may be causally linked to chronic childhood diffuse lung diseases. The profile of these proteins in the alveolar space is unknown in such subjects. METHODS: We analyzed...
Autores principales: | , , , , , , , |
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Formato: | Texto |
Lenguaje: | English |
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BioMed Central
2005
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1224872/ https://www.ncbi.nlm.nih.gov/pubmed/16042774 http://dx.doi.org/10.1186/1465-9921-6-80 |
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author | Griese, Matthias Schumacher, Silja Tredano, Mohammed Steinecker, Manuela Braun, Annika Guttentag, Susan Beers, Michael F Bahuau, Michel |
author_facet | Griese, Matthias Schumacher, Silja Tredano, Mohammed Steinecker, Manuela Braun, Annika Guttentag, Susan Beers, Michael F Bahuau, Michel |
author_sort | Griese, Matthias |
collection | PubMed |
description | BACKGROUND: Abnormalities of the intracellular metabolism of the hydrophobic surfactant proteins SP-B and SP-C and their precursors may be causally linked to chronic childhood diffuse lung diseases. The profile of these proteins in the alveolar space is unknown in such subjects. METHODS: We analyzed bronchoalveolar lavage fluid by Western blotting for SP-B, SP-C and their proforms in children with pulmonary alveolar proteinosis (PAP, n = 15), children with no SP-B (n = 6), children with chronic respiratory distress of unknown cause (cRD, n = 7), in comparison to children without lung disease (n = 15) or chronic obstructive bronchitis (n = 19). RESULTS: Pro-SP-B of 25–26 kD was commonly abundant in all groups of subjects, suggesting that their presence is not of diagnostic value for processing defects. In contrast, pro-SP-B peptides cleaved off during intracellular processing of SP-B and smaller than 19–21 kD, were exclusively found in PAP and cRD. In 4 of 6 children with no SP-B, mutations of SFTPB or SPTPC genes were found. Pro-SP-C forms were identified at very low frequency. Their presence was clearly, but not exclusively associated with mutations of the SFTPB and SPTPC genes, impeding their usage as candidates for diagnostic screening. CONCLUSION: Immuno-analysis of the hydrophobic surfactant proteins and their precursor forms in bronchoalveolar lavage is minimally invasive and can give valuable clues for the involvement of processing abnormalities in pediatric pulmonary disorders. |
format | Text |
id | pubmed-1224872 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2005 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-12248722005-09-22 Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease Griese, Matthias Schumacher, Silja Tredano, Mohammed Steinecker, Manuela Braun, Annika Guttentag, Susan Beers, Michael F Bahuau, Michel Respir Res Research BACKGROUND: Abnormalities of the intracellular metabolism of the hydrophobic surfactant proteins SP-B and SP-C and their precursors may be causally linked to chronic childhood diffuse lung diseases. The profile of these proteins in the alveolar space is unknown in such subjects. METHODS: We analyzed bronchoalveolar lavage fluid by Western blotting for SP-B, SP-C and their proforms in children with pulmonary alveolar proteinosis (PAP, n = 15), children with no SP-B (n = 6), children with chronic respiratory distress of unknown cause (cRD, n = 7), in comparison to children without lung disease (n = 15) or chronic obstructive bronchitis (n = 19). RESULTS: Pro-SP-B of 25–26 kD was commonly abundant in all groups of subjects, suggesting that their presence is not of diagnostic value for processing defects. In contrast, pro-SP-B peptides cleaved off during intracellular processing of SP-B and smaller than 19–21 kD, were exclusively found in PAP and cRD. In 4 of 6 children with no SP-B, mutations of SFTPB or SPTPC genes were found. Pro-SP-C forms were identified at very low frequency. Their presence was clearly, but not exclusively associated with mutations of the SFTPB and SPTPC genes, impeding their usage as candidates for diagnostic screening. CONCLUSION: Immuno-analysis of the hydrophobic surfactant proteins and their precursor forms in bronchoalveolar lavage is minimally invasive and can give valuable clues for the involvement of processing abnormalities in pediatric pulmonary disorders. BioMed Central 2005 2005-07-22 /pmc/articles/PMC1224872/ /pubmed/16042774 http://dx.doi.org/10.1186/1465-9921-6-80 Text en Copyright © 2005 Griese et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Research Griese, Matthias Schumacher, Silja Tredano, Mohammed Steinecker, Manuela Braun, Annika Guttentag, Susan Beers, Michael F Bahuau, Michel Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease |
title | Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease |
title_full | Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease |
title_fullStr | Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease |
title_full_unstemmed | Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease |
title_short | Expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease |
title_sort | expression profiles of hydrophobic surfactant proteins in children with diffuse chronic lung disease |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1224872/ https://www.ncbi.nlm.nih.gov/pubmed/16042774 http://dx.doi.org/10.1186/1465-9921-6-80 |
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