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Article

4-Phenylbutyric acid treatment rescues trafficking and processing of a mutant surfactant protein-C.

American journal of respiratory cell and molecular biology - 1 Sept 2012

Stewart Gareth A, Ridsdale Ross, Martin Emily P, Na Cheng-Lun, Xu Yan, Mandapaka Karunyakanth, Weaver Timothy E

Abstract excerpt

Mutations in the SFTPC gene, encoding surfactant protein-C (SP-C), are associated with interstitial lung disease (ILD). Knowledge of the intracellular fate of mutant SP-C is essential in the design of therapies to correct trafficking/processing of the proprotein, and to prevent the formation of cytotoxic aggregates. We assessed the potential of a chemical chaperone to correct the trafficking and processing of...

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Source

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Identifiers

PMID: 22461427
DOI: 10.1165/rcmb.2012-0003OC
PMCID: PMC3488688

Genes

SFTPC

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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