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Article

SFTPC mutations cause SP-C degradation and aggregate formation without increasing ER stress.

European journal of clinical investigation - 1 Aug 2013

Thurm Tobias, Kaltenborn Eva, Kern Sunčana, Griese Matthias, Zarbock Ralf

Abstract excerpt

BACKGROUND: Mutations in the gene encoding surfactant protein C (SP-C) cause familial and sporadic interstitial lung disease (ILD), which is associated with considerable morbidity and mortality. Unfortunately, effective therapeutic options are still lacking due to a very limited understanding of pathomechanisms. Knowledge of mutant SP-C proprotein (proSP-C) trafficking, processing, intracellular degradation and...

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Source

Identifiers

PMID: 23701443
DOI: 10.1111/eci.12107

Genes

SFTPC

Provenance

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