title: Surfactant protein a in cystic fibrosis: supratrimeric structure and pulmonary outcome.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  Organizational structure of SP-A affects its functional activity and is linked to disease severity in CF.
authors.affiliation: Dr. von Haunersches Kinderspital, University of Munich, Munich, Germany. Matthias.griese@med.uni-muenchen.de
authors.forename: Matthias
authors.initials: M
authors.lastname: Griese
authors.forename: Stephanie
authors.initials: S
authors.lastname: Heinrich
authors.forename: Felix
authors.initials: F
authors.lastname: Ratjen
authors.forename: Michael
authors.initials: M
authors.lastname: Kabesch
authors.forename: Karl
authors.initials: K
authors.lastname: Paul
authors.forename: Manfred
authors.initials: M
authors.lastname: Ballmann
authors.forename: Ernst
authors.initials: E
authors.lastname: Rietschel
authors.forename: Matthias
authors.initials: M
authors.lastname: Kappler
mesh_terms: D000293:Adolescent; D000328:Adult; D018893:Bronchoalveolar Lavage; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry*; D002648:Child; D002850:Chromatography, Gel; D003550:Cystic Fibrosis / Q000378:metabolism* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008168:Lung / Q000378:metabolism; D008297:Male; D037662:Pulmonary Surfactant-Associated Protein A / Q000032:analysis / Q000378:metabolism*; D012720:Severity of Illness Index
chemical_list: D037662:Pulmonary Surfactant-Associated Protein A