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A non-BRICHOS surfactant protein c mutation disrupts epithelial cell function and intercellular signaling

URN to cite this document:
urn:nbn:de:bvb:355-epub-221907
DOI to cite this document:
10.5283/epub.22190
Woischnik, Markus ; Sparr, Christiane ; Kern, Sunčana ; Thurm, Tobias ; Hector, Andreas ; Hartl, Dominik ; Liebisch, Gerhard ; Mulugeta, Surafel ; Beers, Michael F. ; Schmitz, Gerd ; Griese, Matthias
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Date of publication of this fulltext: 26 Sep 2011 09:47


Abstract

Background: Heterozygous mutations of SFTPC, the gene encoding surfactant protein C (SP-C), cause sporadic and familial interstitial lung disease (ILD) in children and adults. The most frequent SFTPC mutation in ILD patients leads to a threonine for isoleucine substitution at position 73 (I73T) of the SP-C preprotein (proSP-C), however little is known about the cellular consequences of SP-C-I73T ...

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