This study developed a mouse model expressing the human SFTPC C121G mutation linked to interstitial lung disease. The model develops spontaneous lung fibrosis and replicates key pathological features of idiopathic pulmonary fibrosis (IPF), including AEC2 cell dysfunction and ER stress.
Expression of the C121G mutation leads to spontaneous, progressive lung fibrosis and impaired lung function, modeling IPF pathology.
Tamoxifen-inducible Sftpc(C121G) Knockin mouse model with AEC2-specific expression of the mutation.
Idiopathic pulmonary fibrosis, Lung fibrosis, ER stress, Alveolar epithelium
Knockin, Tamoxifen-inducible, Point mutation, AEC2-specific expression, Rosa26
From model design to experimental results
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