Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences.
Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences.
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DOI：
10.1155/2016/5258727
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发表时间：
2016
影响因子：
--
通讯作者：
Reeves EP
中科院分区：
文献类型：
--
作者：
Lavelle GM;White MM;Browne N;McElvaney NG;Reeves EP
囊性纤维化（CF）是由囊性纤维化跨膜传导调节因子（CFTR）基因突变引起的。由此产生的特征性离子运输缺陷导致纤毛粘膜清除率降低、细菌定植和慢性中性粒细胞主导的炎症。尽管每一种动物模型都有其固有的局限性，但通过动物模型的生成已经获得了有关该病病理生理学的许多知识。由于某些小鼠模型无法再现人类疾病的表型表现，因此开始产生更大的动物来研究CF，包括猪和雪貂。这篇综述将总结三种动物模型的基本表型，并描述这些动物研究对我们目前对CF的理解的贡献。
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The resultant characteristic ion transport defect results in decreased mucociliary clearance, bacterial colonisation, and chronic neutrophil-dominated inflammation. Much knowledge surrounding the pathophysiology of the disease has been gained through the generation of animal models, despite inherent limitations in each. The failure of certain mouse models to recapitulate the phenotypic manifestations of human disease has initiated the generation of larger animals in which to study CF, including the pig and the ferret. This review will summarise the basic phenotypes of three animal models and describe the contributions of such animal studies to our current understanding of CF.