Surfactant protein a in cystic fibrosis: supratrimeric structure and pulmonary outcome

The state of oligomerization of surfactant associated protein-A (SP-A) monomers differs between individuals. This likely affects SP-A's functional properties and could thereby influence clinical status in patients with lung diseases. In this study we focus on SP-A structure in cystic fibrosis (...

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Veröffentlicht in:PloS one 2012-12, Vol.7 (12), p.e51050-e51050
Hauptverfasser: Griese, Matthias, Heinrich, Stephanie, Ratjen, Felix, Kabesch, Michael, Paul, Karl, Ballmann, Manfred, Rietschel, Ernst, Kappler, Matthias
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container_title PloS one
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creator Griese, Matthias
Heinrich, Stephanie
Ratjen, Felix
Kabesch, Michael
Paul, Karl
Ballmann, Manfred
Rietschel, Ernst
Kappler, Matthias
description The state of oligomerization of surfactant associated protein-A (SP-A) monomers differs between individuals. This likely affects SP-A's functional properties and could thereby influence clinical status in patients with lung diseases. In this study we focus on SP-A structure in cystic fibrosis (CF) compared to both healthy subjects and disease controls. SP-A composition and function were assessed in both bronchoalveolar lavage (BAL) fluid and serum of 46 CF patients with mild disease, 25 patients with chronic bronchitis and 22 healthy subjects by gel chromatography and a functional agglutination assay. Relation of SP-A agglutination ability to disease severity of the subjects was explored. SP-A was present in seven major oligomeric forms with the majority of SP-A being structurally organized as complex oligomeric forms. More complex oligomeric forms were associated with better SP-A function with regard to its agglutination ability. These forms were more frequently observed in BAL than in serum, but there were no differences between disease groups. In CF patients, more complex forms of SP-A were associated with better lung function. Organizational structure of SP-A affects its functional activity and is linked to disease severity in CF.
doi_str_mv 10.1371/journal.pone.0051050
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subjects Adolescent
Adult
Agglutination
Alveoli
Biology
Bronchitis
Bronchoalveolar Lavage
Bronchoalveolar Lavage Fluid - chemistry
Bronchus
Child
Chromatography
Chromatography, Gel
Control methods
Cystic fibrosis
Cystic Fibrosis - metabolism
Cystic Fibrosis - physiopathology
Disease control
Female
Gel chromatography
Humans
Inflammation
Lung - metabolism
Lung diseases
Male
Medical research
Medicine
Microorganisms
Monomers
Oligomerization
Oligomers
Organizational structure
Patients
Protein A
Proteins
Pulmonary Surfactant-Associated Protein A - analysis
Pulmonary Surfactant-Associated Protein A - metabolism
Respiratory function
Severity of Illness Index
Structure-function relationships
Surface active agents
Surfactant protein A
Surfactants
title Surfactant protein a in cystic fibrosis: supratrimeric structure and pulmonary outcome
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