Primary bronchial epithelial cell culture from people with cystic fibrosis (CF) - a model to study CF lung disease

在英国，超过7000人患有囊性纤维化（CF），使其成为最常见的限制生命的遗传性疾病。CF的死亡和疾病主要是由于肺部疾病的进展。本病的特点是严重的炎症，特别是涉及中性粒细胞脓液细胞和结构性气道损伤。中性粒细胞炎症是CF的早期发现之一，发生在明显感染之前的幼儿。白细胞介素(IL)-17是一种由机体产生的化学介质，近年来作为中性粒细胞-脓细胞在一系列炎症性疾病中的驱动因子引起了人们的极大兴趣。在CF中，有证据表明当患者身体不适时，痰中IL-17浓度升高。我们是欧洲第一个在移植时从CF患者的肺中培养细胞的宝贵实验资源的团队。使用我们的CF肺部疾病新模型的第一个研究将集中在IL-17的作用上。对炎症过程的进一步了解可能会导致新的治疗方法能够减轻炎症和肺损伤，这是CF的一个问题。

Over 7000 people have cystic fibrosis (CF) in the United Kingdom making it the most common life-limiting inherited disease. Death and illness in CF are largely due to progressive lung disease. The disease is characterised by severe inflammation, particularly involving neutrophilic pus cells, and structural airway damage. Neutrophilic inflammation is one of the earliest findings in CF occurring in young children prior to obvious infection. Interleukin(IL)-17, a chemical mediator produced by the body, has attracted great interest recently as a driver of neutrophil-pus cells in a range of inflammatory diseases. In CF there is evidence that concentrations of IL-17 rise in sputum when patients become unwell.We are the first group in Europe to establish the invaluable experimental resource of culturing cells from the lungs of actual people with CF at the time of transplantation. The first studies using our new model of CF lung disease will focus on the role of IL-17. An improved understanding of the inflammatory process may lead to new treatments capable of alleviating the inflammation and lung damage that is such a problem in CF.