In a Sftpc^C121G point mutation mouse model of pulmonary fibrosis, alveolar type II (AT2) epithelial cells adopt an aberrant intermediate (basaloid‑like) phenotype. These cells exhibit a profibrotic secretome (notably TGF‑β signaling) and engage fibroblasts via a ligand–receptor interactome that activates fibroblasts. Human iPSC‑derived SFTPC‑mutant AT2s recapitulate the intermediate phenotype under loss of progenitor signaling plus TGF‑β stimulation. These aberrant intermediate epithelial cells drive fibroblast pathogenic activation in preclinical fibrosis models.
Aberrant intermediate epithelial cells appear early, share transcriptional features with human fibrotic basaloid cells, secrete profibrotic factors, activate fibroblasts ex vivo, contribute to lung fibrosis progression in vivo
Sftpc^C121G point mutation knockin mouse (AT2-specific mutant surfactant protein C)
Pulmonary fibrosis, epithelial–mesenchymal crosstalk, profibrotic signaling, TGF‑β pathway, lung regeneration failure
Knockin of Sftpc C121G mutant allele in AT2 lineage, tamoxifen-induction, single-cell RNA sequencing, ligand–receptor interactome analysis, epithelial–fibroblast co-culture assays, human iPSC modeling
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