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Article

Meckel-Gruber syndrome protein MKS3 is required for endoplasmic reticulum-associated degradation of surfactant protein C.

The Journal of biological chemistry - 27 Nov 2009

Wang Mei, Bridges James P, Na Cheng-Lun, Xu Yan, Weaver Timothy E

Abstract excerpt

Autosomal dominant mutations in the SFTPC gene are associated with idiopathic pulmonary fibrosis, a progressive lethal interstitial lung disease. Mutations that cause misfolding of the encoded proprotein surfactant protein C (SP-C) trigger endoplasmic reticulum (ER)-associated degradation, a path...

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Source

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Identifiers

PMID: 19815549
DOI: 10.1074/jbc.M109.034371
PMCID: PMC2785181

Genes

SFTPC
TMEM67

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-19815549-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

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Meckel-Gruber syndrome protein MKS3 is required for endoplasmic reticulum-associated degradation of surfactant protein C.PMID 19815549Read ↗

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