Article
Elevated xylosyltransferase I activities in pseudoxanthoma elasticum (PXE) patients as a marker of stimulated proteoglycan biosynthesis.
Journal of molecular medicine (Berlin, Germany) - 1 Dec 2005
Götting Christian, Hendig Doris, Adam Alexandra, Schön Sylvia, Schulz Veronika, Szliska Christiane, Kuhn Joachim, Kleesiek Knut
Abstract excerpt
Pseudoxanthoma elasticum (PXE) is a hereditary disorder of the connective tissue characterized by extracellular matrix alterations with elastin fragmentation and excessive proteoglycan deposition. Xylosyltransferase I (XT-I, E.C. 2.4.2.26) is the initial enzyme in the biosynthesis of the glycosaminoglycan chains in proteoglycans and has been shown to be a marker of tissue remodeling processes. Here, we...
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