Dr. von Haunersches Kinderspital, University of Munich, Munich, Germany.
PLoS One. 2012;7(12):e51050. doi: 10.1371/journal.pone.0051050. Epub 2012 Dec 7.
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.
表面活性剂相关蛋白 A（SP-A）单体的聚集状态在个体之间存在差异。这可能会影响 SP-A 的功能特性，并因此影响肺部疾病患者的临床状况。在这项研究中，我们重点研究了囊性纤维化（CF）中 SP-A 的结构，将其与健康受试者和疾病对照组进行了比较。
通过凝胶色谱法和功能凝集试验，评估了 46 例轻度 CF 患者、25 例慢性支气管炎患者和 22 例健康受试者的支气管肺泡灌洗液（BAL）和血清中的 SP-A 组成和功能。探讨了 SP-A 凝集能力与受试者疾病严重程度的关系。
SP-A 存在 7 种主要的寡聚形式，大多数 SP-A 以复杂的寡聚形式存在。在凝集能力方面，更复杂的寡聚形式与更好的 SP-A 功能相关。这些形式在 BAL 中比在血清中更常见，但在疾病组之间没有差异。在 CF 患者中，更复杂的 SP-A 形式与更好的肺功能相关。
SP-A 的组织结构影响其功能活性，并与 CF 中的疾病严重程度相关。
