肺泡蛋白沉积症动物模型的构建及应用  

Establishment and application of animal models of pulmonary alveolar proteinosis

作　　者：蔡嘉慧 宋新宇 罗钰龙 李时悦[1] Cai Jiahui;Song Xinyu;Luo Yulong;Li Shiyue(The First Affiliated Hospital of Guangzhou Medical University,National Centre for Respiratory Medicine,Guangzhou Institute of Respiratory Health,State Key Laboratory of Respiratory Disease,National Clinical Research Center for Respiratory Disease Bioland Laboratory,Guangzhou Regenerative Medicine and Health Guangdong Laboratory,Guangzhou 510005,China)

机构地区：[1]广州医科大学附属第一医院国家呼吸医学中心广州呼吸健康研究院呼吸疾病国家重点实验室国家呼吸系统疾病临床医学中心生物岛实验室广州再生医学与健康广东省实验室,510005

出　　处：《中华结核和呼吸杂志》2022年第1期106-110,共5页Chinese Journal of Tuberculosis and Respiratory Diseases

基　　金：生物岛实验室临床创新研究项目(2018GZR0201002);广州呼吸健康研究院自主立项课题项目(ZNSA-2020013)。

摘　　要：肺泡蛋白沉积症(PAP)是一种呼吸系统罕见病,研究进展缓慢。动物模型是开展各类基础研究的有效工具,目前的动物模型均是根据粒细胞-巨噬细胞集落刺激因子(GM-CSF)信号作用障碍和肺泡内环境稳态失衡这两个主要的发病机制而建立,应用研究集中在PAP的治疗策略上。现有的PAP动物模型均不能完全反映人PAP疾病发展,需进一步开发和改进,为临床实践提供依据。Pulmonary alveolar proteinosis(PAP)is a rare respiratory disease,but this disease has slow research progress.Animal model is an effective tool for basic research.Current PAP animal models are based on the main pathogenesis of granulocyte-macrophage colony stimulation factor(GM-CSF)signal disorder and environmental homeostasis imbalance in the alveoli.Application researches focus on the treatment strategies of PAP.The existing PAP animal models cannot fully reflect to the development of human PAP,which should be further developed and improved to provide the basis for clinical practice.

关 键 词：肺泡蛋白沉积症 罕见病 内环境稳态 动物模型 信号作用 临床实践 治疗策略 呼吸系统 

分 类 号：R-332[医药卫生] R56