Regnier Agathe, Dannhoffer Luc, Blouquit-Laye Sabine, Bakari Mariama, Naline Emmanuel, Chinet Thierry
AP-HP, Hôpital Ambroise Paré, Service d'Anatomie Pathologique, 92104 Boulogne, France.
Hum Pathol. 2008 Mar;39(3):368-76. doi: 10.1016/j.humpath.2007.06.020. Epub 2007 Nov 28.
The determination of the expression of cystic fibrosis transmembrane conductance regulator (CFTR) in the lung is essential for a full understanding of the normal lung physiology and the pathogenesis of the lung disease in cystic fibrosis (CF). However, studies on the expression of CFTR in the distal adult human lung have yielded conflicting results despite functional evidence of expression of CFTR in bronchiolar and alveolar epithelial cells. We used 2 high-affinity monoclonal anti-CFTR antibodies, MAb24-1 and MAb13-1, to determine the expression of CFTR in samples of bronchiolar and alveolar tissues obtained from the same non-CF individuals. CFTR immunostaining was detected in the epithelium of bronchiolar and alveolar tissues. The staining pattern was similar with both antibodies. In bronchioles, CFTR labeling was present mostly in ciliated cells; in alveoli, CFTR labeling was detected in both type I and type II cells. We conclude that CFTR is expressed in human bronchiolar and alveolar epithelial cells. The potential importance of CFTR expression in alveoli should be further investigated, particularly with respect to the CF lung disease and the physiology of the alveolar region.
确定囊性纤维化跨膜传导调节因子（CFTR）在肺中的表达对于全面了解正常肺生理以及囊性纤维化（CF）肺病的发病机制至关重要。然而，尽管有功能证据表明CFTR在细支气管和肺泡上皮细胞中表达，但关于CFTR在成人远端肺中的表达研究结果却相互矛盾。我们使用两种高亲和力单克隆抗CFTR抗体MAb24 - 1和MAb13 - 1，来确定从相同非CF个体获得的细支气管和肺泡组织样本中CFTR的表达。在细支气管和肺泡组织的上皮中检测到CFTR免疫染色。两种抗体的染色模式相似。在细支气管中，CFTR标记主要存在于纤毛细胞中；在肺泡中，在I型和II型细胞中均检测到CFTR标记。我们得出结论，CFTR在人细支气管和肺泡上皮细胞中表达。CFTR在肺泡中表达的潜在重要性应进一步研究，特别是关于CF肺病和肺泡区域的生理学方面。
