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Title:Matrix Gla protein (MGP) is involved in elastic fiber calcification in the dermis of Pseudoxanthoma Elasticum (PXE) patients.
Authors:Dealba, Gheduzzi (Author)
Boraldi, Federica (Author)
Giulia, Annovi (Author)
Chiara, Paolinelli Devincenzi (Author)
Leon J, Schurgers (Author)
Cees, Vermeer (Author)
Daniela, Quaglino (Author)
Ivonne, Pasquali Ronchetti (Author)
et al.
Files:This document has no files. This document may have a phisical copy in the library of the organization, check the status via COBISS. Link is opened in a new window
Language:English
Work type:Not categorized (r6)
Tipology:1.01 - Original Scientific Article
Organization:UNG - University of Nova Gorica
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
Year of publishing:2007
Number of pages:998-1008
Numbering:87
COBISS_ID:5424635 Link is opened in a new window
URN:URN:SI:UNG:REP:HP9EHLDU
License:CC BY-NC-ND 4.0
This work is available under this license: Creative Commons Attribution Non-Commercial No Derivatives 4.0 International
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Title:Laboratory Investigation
Year of publishing:2007

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