Semaniakou Anna, Croll Roger P, Chappe Valerie
Department of Physiology and Biophysics, Faculty of Medicine, Dalhousie University, Halifax, NS, Canada.
Front Pharmacol. 2019 Jan 4;9:1475. doi: 10.3389/fphar.2018.01475. eCollection 2018.
Our understanding of the multiorgan pathology of cystic fibrosis (CF) has improved impressively during the last decades, but we still lack a full comprehension of the disease progression. Animal models have greatly contributed to the elucidation of specific mechanisms involved in CF pathophysiology and the development of new therapies. Soon after the cloning of the CF transmembrane conductance regulator () gene in 1989, the first mouse model was generated and this model has dominated CF research ever since. Nonetheless, the failure of murine models to mirror human disease severity in the pancreas and lung has led to the generation of larger animal models such as pigs and ferrets. The following review presents and discusses data from the current animal models used in CF research.
在过去几十年里，我们对囊性纤维化（CF）多器官病理的理解有了显著提高，但我们仍未完全理解该疾病的进展。动物模型对阐明CF病理生理学中涉及的特定机制以及新疗法的开发做出了巨大贡献。1989年CF跨膜传导调节因子（）基因克隆后不久，首个小鼠模型就被构建出来，此后该模型一直主导着CF研究。尽管如此，小鼠模型未能反映出人类胰腺和肺部疾病的严重程度，这导致了诸如猪和雪貂等更大动物模型的产生。以下综述展示并讨论了目前用于CF研究的动物模型的数据。
