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Article

Novel insights into surfactant protein C trafficking revealed through the study of a pathogenic mutant.

The European respiratory journal - 1 Jan 2022

Dickens Jennifer A, Rutherford Eimear N, Abreu Susana, Chambers Joseph E, Ellis Matthew O, van Schadewijk Annemarie, Hiemstra Pieter S, Marciniak Stefan J

Abstract excerpt

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 pathogenic mutant, I73T, mislocalises to the alveolar epithelial cell plasma membrane and displays a toxic gain of...

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Source

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Identifiers

PMID: 34049951
DOI: 10.1183/13993003.00267-2021
PMCID: PMC8792467

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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