Dealba Gheduzzi (Author), Federica Boraldi (Author), Giulia Annovi (Author), Chiara Paolinelli DeVincenzi (Author), Leon J. Schurgers (Author), Cees Vermeer (Author), Daniela Quaglino (Author), Ivonne Pasquali Ronchetti (Author)

Abstract

Mature MGP (Matrix g-carboxyglutamic acid protein) is known to inhibit soft connective tissues calcification. We investigated its possible involvement in pseudoxanthoma elasticum (PXE), a genetic disorder whose clinical manifestations are due to mineralization of elastic fibers. PXE patients have lower serum concentration of total MGP compared to controls (Po0.001). Antibodies specific for the noncarboxylated (Glu-MGP) and for the g-carboxylated (Gla-MGP) forms of MGP were assayed on ultrathin sections of dermis from controls and PXE patients. Normal elastic fibers in controls and patients were slightly positive for both forms of MGP, whereas Gla-MGP was more abundant within control’s than within patient’s elastic fibers (Po0.001). In patients’ calcified elastic fibers, Glu-MGP intensively colocalized with mineral precipitates, whereas Gla-MGP precisely localized at the mineralization front. Data suggest that MGP is present within elastic fibers and is associated with calcification of dermal elastic fibers in PXE.

Keywords

calcification;dermal fibroblast;elastic fiber;human skin;MGP;pseudoxanthoma elasticum;

Data

Language: English
Year of publishing:
Typology: 1.01 - Original Scientific Article
Organization: UNG - University of Nova Gorica
UDC: 616
COBISS: 5424635 Link will open in a new window
ISSN: 0023-6837
Views: 2586
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Other data

URN: URN:SI:UNG
Type (COBISS): Not categorized
Pages: str. 998-1008
Volume: ǂVol. ǂ87
Issue: ǂiss. ǂ10
Chronology: Oct. 2007
DOI: 10.1038/labinvest.3700667
ID: 11192838