CFTR ion transport deficiency primes the epithelium for partial epithelial-mesenchymal transition in cystic fibrosis
Altogether, these findings demonstrate that the loss of CFTR ion transport, despite the physical presence of (nonfunctional) CFTR at the PM, is enough to trigger partial EMT. However, the severity of the EMT phenotype worsens when CFTR is absent from the PM while also increasing susceptibility to TGF-β1-triggered EMT. Moreover, CFTRm only partially reverse this CF EMT state, indicating that full epithelial integrity will likely require targeting additional factors.
Authors
- Cláudia S. Rodrigues (ORCID: https://orcid.org/0000-0002-9922-1879)
- Ines Pankonien (ORCID: https://orcid.org/0000-0002-4622-7521)
- Carlos M. Farinha (ORCID: https://orcid.org/0000-0002-5467-1710)
- Violeta Railean (ORCID: https://orcid.org/0000-0003-3713-7089)
- Raquel Torres
- Sofia S. Ramalho (ORCID: https://orcid.org/0000-0002-7554-9802)
- Margarida D. Amaral (ORCID: https://orcid.org/0000-0002-0828-8630)
- Maurizio Dal Canto
Institutions
- University of Lisbon (PT)
Publication Details
- Journal
- Frontiers in Pharmacology
- Published
- 2025-08-20
- DOI
- https://doi.org/10.3389/fphar.2025.1655479
- Primary Topic
- Cystic Fibrosis Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Ministério da Ciência, Tecnologia e Ensino Superior
- Horizon 2020 Framework Programme
- Fundação para a Ciência e a Tecnologia