2.
Matrix Gla protein (MGP) is involved in elastic fiber calcification in the dermis of Pseudoxanthoma Elasticum (PXE) patients.Gheduzzi Dealba,
Federica Boraldi,
Annovi Giulia,
Paolinelli Devincenzi Chiara,
Schurgers Leon J,
Vermeer Cees,
Quaglino Daniela,
Pasquali Ronchetti Ivonne, 2007, original scientific article
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
Published in RUNG: 22.07.2019; Views: 4690; Downloads: 0
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