Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease characterized by a continual loss of pulmonary function. Its 5-year survival rate is approximately 20%. Despite the approval of two drugs, pirfenidone and nintedanib, to slow the progression of the disease in certain IPF patients, there is still no effective therapy. No natural models can fully recapitulate all features of human IPF.
The most widely used experimental model involves intratracheal instillation of bleomycin, validated through collagen expression levels and CT image analysis. However, the bleomycin-induced mouse model presents challenges due to the uneven severity of fibrosis caused by endotracheal administration, whether surgical or nonsurgical. This inconsistency leads to a "blind men touching an elephant" phenomenon during the collection of lung tissue samples. The limitations of current animal models contribute to the high failure rate of clinical trials for IPF.
The goal of this collection is to establish the use of advanced animal models that reproduce key known features of the disease, such as lung transplantation-induced models, humanized animal models, CTD-ILD-related fibrosis models, and unilateral fibrosis models. These improved models will provide more accurate and easily assessable platforms for evaluating drugs and treatment strategies for pulmonary fibrosis.
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2025
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Qian Jiang*1, Dongmei Wen*1, Fang Peng*2, Wei Hong3, Xin Fu3, Yuanhui Xiong1, Chongyuan Ren1, Xuanyi Li1, Dansha Zhou1,4, Jian Wang1,4, Yuqin Chen1
1State Key Laboratory of Respiratory Diseases, National Center for Respiratory Medicine, Guangdong Key Laboratory of Vascular Diseases, National Clinical Research Center for Respiratory Diseases, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital of Guangzhou Medical University, 2Department of Critical Care Medicine, The Third Affiliated Hospital of Guangzhou Medical University, 3GMU-GIBH Joint School of Life Sciences, Guangzhou Medical University, 4Guangzhou laboratory, Guangzhou International Bio Island
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2025
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Xihui Huang*1,2, Shu Xia*1, Yiqian Yang*1, Chengcheng Lei*3, Penghui Yang1, Shuang Li1, Jianyi Niu1, Jian Wu3, Qun Luo1, Zhenli Fu3
1State Key Laboratory of Respiratory Diseases, National Clinical Research Center for Respiratory Diseases, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital of Guangzhou Medical University, 2Guangzhou National Laboratory, 3Second Department of Elderly Respiratory, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangdong Provincial Geriatric Institute
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Cited by 1
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2025
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Hailin Liao*1, Xiaohua Wang*1,2, Yi Lu*1, Wenshan Zhong1, Jiang Shi1, Xihui Huang1, Xu Chen1, Guilin Li1, Penghui Yang2, Chunrong Ju1,2
1State Key Laboratory of Respiratory Diseases, National Clinical Research Center for Respiratory Diseases, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital of Guangzhou Medical University, 2Department of Organ Transplantation, the First Affiliated Hospital of Guangzhou Medical University
Preliminary Study on Induction and Imaging Evaluation of Idiopathic Pulmonary Fibrosis in Macaca fascicularis
Junhua Rao*1,
Jianhuan Chen*2,
jiangmei Gao1
1Institute of Zoology, Guangdong Academy of Sciences, 105 Xingang Rd. West, Guangzhou, China ,
2Laboratory of Genomic and Precision Medicine, Wuxi School of Medicine, Jiangnan University, 1800 Lihu Avenue, Wuxi, Jiangsu 214122, China.
Establishment and evaluation of an in vitro human-based advanced model of pulmonary fibrosis
Xihui Huang1,
Penghui Yang*1
1State Key Laboratory of Respiratory Diseases, National Clinical Research Center for Respiratory Diseases, Guangzhou Institute of Respiratory Health, the First Affiliated Hospital of Guangzhou Medical University