Article
Mutant fibrillin 1 from tight skin mice increases extracellular matrix incorporation of microfibril-associated glycoprotein 2 and type I collagen.
Arthritis and rheumatism - 1 Mar 2004
Lemaire Raphael, Farina Guiseppina, Kissin Eugene, Shipley J Michael, Bona Constantine, Korn Joseph H, Lafyatis Robert
Abstract excerpt
OBJECTIVE: Skin fibrosis in the TSK mouse, a model of skin fibrosis seen in systemic sclerosis (SSc), is caused by a large in-frame duplication in the Fbn1 gene, tsk-Fbn1. We investigated whether tsk-Fbn1 might cause dermal fibrosis by affecting Fbn1 and associated extracellular matrices. We also studied whether deposition of microfibril-associated glycoprotein 2 (MAGP-2), a protein that is associated with...
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