Henke Markus O, Renner Armin, Huber Rudolf M, Seeds Michael C, Rubin Bruce K
Department of Pulmonary Medicine, Philipps-University Marburg, Germany.
Am J Respir Cell Mol Biol. 2004 Jul;31(1):86-91. doi: 10.1165/rcmb.2003-0345OC. Epub 2004 Feb 26.
Cystic fibrosis (CF) is characterized by progressive airway obstruction. Although it has been postulated that this is due in part to mucus hypersecretion, there are no published data showing an increase in the gel-forming mucins MUC5AC or MUC5B in CF secretions. We used confocal microscopy to assess the amount of mucin-like glycoprotein and DNA in CF sputum and found more mucin in bronchitis sputum and a much greater amount of DNA in CF sputum. We then used antibodies to MUC5AC and MUC5B with Western gels and dot-blot to quantify mucin in sputum from 12 patients with CF and 11 subjects without lung disease. There was a 70% decrease in MUC5B and a 93% decrease in MUC5AC in CF sputum (P < 0.005 for both). We conclude that the vol/vol concentration of MUC5AC and MUC5B are decreased in the CF airways relative to normal mucus. This may be due to a relative increase in other components of sputum in the CF airway or to a primary defect in mucin secretion in CF.
囊性纤维化(CF)的特征是进行性气道阻塞。尽管据推测这部分归因于黏液分泌过多,但尚无已发表的数据表明CF分泌物中形成凝胶的黏蛋白MUC5AC或MUC5B有所增加。我们使用共聚焦显微镜评估CF痰液中黏蛋白样糖蛋白和DNA的含量,发现支气管炎痰液中的黏蛋白更多,而CF痰液中的DNA含量则多得多。然后,我们使用针对MUC5AC和MUC5B的抗体进行蛋白质免疫印迹和斑点印迹,以量化12例CF患者和11例无肺部疾病受试者痰液中的黏蛋白。CF痰液中MUC5B减少了70%,MUC5AC减少了93%(两者P均<0.005)。我们得出结论,相对于正常黏液,CF气道中MUC5AC和MUC5B的体积/体积浓度降低。这可能是由于CF气道中痰液其他成分的相对增加,或CF中黏蛋白分泌的原发性缺陷所致。