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1 Children's Clinic of Ludwig-Maximilians-University, Munich, Germany. 2 Children's Clinic, Inselspital, University of Bern, Switzerland
CORRESPONDENCE: M. Griese, Kinderklinik und Kinderpoliklinik im Dr. von Haunerschen Kinderspital, Ludwig-Maximilians University, Pettenkoferstrasse 8a, Munich, Germany, D-80336. Fax: 49 8951603477
Keywords: bronchoalveolar lavage, cystic fibrosis, proteome, surfactant protein A
Received: August 30, 2000
Accepted December 8, 2000
This project was supported by a grant from the Wilhelm Sander Stiftung (Gr 93.002.1/2).
Inflammation and proteolytic processes play an important role in the progression of cystic fibrosis (CF) lung disease. The goal of this study was to describe bronchoalveolar lavage fluid (BALF) protein pattern of CF patients in comparison to controls and to assess if there is proteolytic degradation of surfactant protein A (SP-A), an important innate host defence component of the lungs.
BALFs from 17 clinically stable CF patients and from eight healthy children were separated by two-dimensional gel electrophoresis. Silver staining was used to show BALF proteins and Western blotting to detect SP-A isoforms.
In CF, BALF proteins of a low molecular weight
Proteolytic damage to surfactant protein A and significant changes of normal bronchoalveolar lavage fluid proteins occur in lungs of cystic fibrosis patients. Identification of altered bronchoalveolar lavage fluid proteins may give new insights into pathogenic mechanisms and provide new targets for therapy.
20 kD were more abundant than in controls. Various proteins were seen in CF which were not present in controls and vice versa. Degradation of SP-A was present in 15 of 17 CF BALFs but in none of the controls, in contrast polymeric isoforms were seen in all controls and in four of 17 CF patients.
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