Article
Mutations linked to interstitial lung disease can abrogate anti-amyloid function of prosurfactant protein C.
The Biochemical journal - 1 Dec 2008
Nerelius Charlotte, Martin Emily, Peng Siwei, Gustafsson Magnus, Nordling Kerstin, Weaver Timothy, Johansson Jan
Abstract excerpt
The newly synthesized proSP-C (surfactant protein C precursor) is an integral ER (endoplasmic reticulum) membrane protein with a single metastable polyvaline alpha-helical transmembrane domain that comprises two-thirds of the mature peptide. More than 20 mutations in the ER-lumenal CTC (C-terminal domain of proSP-C), are associated with ILD (interstitial lung disease), and some of the mutations cause...
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