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Novel insights into surfactant protein C trafficking revealed through the study of a pathogenic mutant
BACKGROUND: Alveolar epithelial cell dysfunction plays an important role in the pathogenesis of idiopathic pulmonary fibrosis (IPF), but remains incompletely understood. Some monogenic forms of pulmonary fibrosis are associated with expression of mutant surfactant protein C (SFTPC). The commonest pa...
Autores principales: | Dickens, Jennifer A., Rutherford, Eimear N., Abreu, Susana, Chambers, Joseph E., Ellis, Matthew O., van Schadewijk, Annemarie, Hiemstra, Pieter S., Marciniak, Stefan J. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
European Respiratory Society
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8792467/ https://www.ncbi.nlm.nih.gov/pubmed/34049951 http://dx.doi.org/10.1183/13993003.00267-2021 |
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