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

Preview

PDF
(1MB)

Date of publication of this fulltext: 26 Sep 2011 09:47

Alternative links to fulltext:Pubmed DOI

Details

Preview

Indexed in

Export bibliographical data

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 ...