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<p>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, <i>Sftpc</i><sup><i>−/−</i></sup> mice, to provide proof-of-principle for this approach. <i>Sftpc</i><sup><i>−/−</i></sup> 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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年1月20日

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  • Camilla Predella,
  • Lauren Lapsley,
  • Keyue Ni,
  • Tania A. Thimraj,
  • Hsiao-Yun Liu,
  • John W. Murray,
  • Joshua E. Motelow,
  • Hans-Willem Snoeck,
  • Stephan W. Glasser,
  • Anjali Saqi,
  • N. Valerio Dorrello

摘要

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.