Rogers Christopher S, Abraham William M, Brogden Kim A, Engelhardt John F, Fisher John T, McCray Paul B, McLennan Geoffrey, Meyerholz David K, Namati Eman, Ostedgaard Lynda S, Prather Randall S, Sabater Juan R, Stoltz David Anthony, Zabner Joseph, Welsh Michael J
Department of Internal Medicine, Roy J. Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA.
Am J Physiol Lung Cell Mol Physiol. 2008 Aug;295(2):L240-63. doi: 10.1152/ajplung.90203.2008. Epub 2008 May 16.
Airway disease currently causes most of the morbidity and mortality in patients with cystic fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing novel therapeutic strategies have been hampered by the limitations of current models. Although the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) has been targeted in mice, CF mice fail to develop lung or pancreatic disease like that in humans. In many respects, the anatomy, biochemistry, physiology, size, and genetics of pigs resemble those of humans. Thus pigs with a targeted CFTR gene might provide a good model for CF. Here, we review aspects of porcine airways and lung that are relevant to CF.
目前，气道疾病是囊性纤维化（CF）患者发病和死亡的主要原因。然而，当前模型的局限性阻碍了对CF肺部疾病发病机制的理解以及新型治疗策略的开发。尽管在小鼠中已对编码囊性纤维化跨膜传导调节因子（CFTR）的基因进行了靶向研究，但CF小鼠并未像人类那样出现肺部或胰腺疾病。在许多方面，猪的解剖结构、生物化学、生理学、体型和遗传学与人类相似。因此，具有靶向CFTR基因的猪可能为CF提供一个良好的模型。在此，我们综述了与CF相关的猪气道和肺部的各个方面。
