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Title:Matrix Gla protein (MGP) is involved in elastic fiber calcification in the dermis of Pseudoxanthoma Elasticum (PXE) patients.
Authors:ID Dealba, Gheduzzi, University of Modena and Reggio Emilia (Author)
ID Boraldi, Federica, University of Modena and Reggio Emilia (Author)
ID Giulia, Annovi, University of Modena and Reggio Emilia (Author)
ID Chiara, Paolinelli Devincenzi, University of Modena and Reggio Emilia (Author)
ID Leon J, Schurgers, University of Maasctricht (Author)
ID Cees, Vermeer, UNiversity of Maastricht (Author)
ID Daniela, Quaglino, University of Modena and Reggio Emilia (Author)
ID Ivonne, Pasquali Ronchetti, University of Modena and Reggio Emilia (Author), et al.
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Language:English
Work type:Not categorized
Typology: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
PID:20.500.12556/RUNG-4661-590a01f0-8ed3-b12c-15e6-2ba17feda471 New window
COBISS.SI-ID:5424635 New window
NUK URN:URN:SI:UNG:REP:HP9EHLDU
Publication date in RUNG:22.07.2019
Views:3226
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Record is a part of a journal

Title:Laboratory Investigation
Year of publishing:2007

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License:CC BY-NC-ND 4.0, Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
Link:http://creativecommons.org/licenses/by-nc-nd/4.0/
Description:The most restrictive Creative Commons license. This only allows people to download and share the work for no commercial gain and for no other purposes.
Licensing start date:19.07.2019

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