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。
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abstractsPulmonary 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.
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