Engraftment of wild-type alveolar type II epithelial cells in surfactant protein C deficient mice

Childhood interstitial lung disease (chILD) secondary to pulmonary surfactant deficiency is a devastating chronic lung disease in children. Clinical presentation includes mild to severe respiratory failure and fibrosis. There is no specific treatment, except lung transplantation, which is hampered by a severe shortage of donor organs, especially for young patients. Repair of lungs with chILD represents a longstanding therapeutic challenge but cell therapy is a promising strategy. As surfactant is produced by alveolar epithelial type II (ATII) cells, engraftment with normal or gene-corrected ATII cells might provide an avenue to cure. Here, we used a chILD disease-like model, Sftpc−/− mice, to provide proof-of-principle for this approach. Sftpc−/− mice developed chronic interstitial lung disease with age and were hypersensitive to bleomycin. We could engraft wild-type ATII cells after low dose bleomycin conditioning. Transplanted ATII cells produced mature SPC and attenuated bleomycin-induced lung injury up to two months post-transplant. This study demonstrates that partial replacement of mutant ATII cells can promote lung repair in a mouse model of chILD-like disease.

• Publication year

  2026

• Venue

  npj Regenerative Medicine

• Publication date

  2026-01-20

• Fields of study

  Medicine

• Identifiers
  DOI 10.1038/s41536-026-00455-0PMID 41554733PMCID 12905338
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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