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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...

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Detalles Bibliográficos
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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2022
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