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1.
Matrix Gla protein (MGP) is involved in elastic fiber calcification in the dermis of Pseudoxanthoma Elasticum (PXE) patients.
Federica Boraldi, Annovi Giulia, Paolinelli Devincenzi Chiara, Schurgers Leon J, Vermeer Cees, Quaglino Daniela, Pasquali Ronchetti Ivonne, Gheduzzi Dealba, 2007, izvirni znanstveni članek

Opis: 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.
Najdeno v: ključnih besedah
Povzetek najdenega: ...calcification, dermal fibroblast, elastic fiber, human skin, MGP, pseudoxanthoma elasticum...
Ključne besede: calcification, dermal fibroblast, elastic fiber, human skin, MGP, pseudoxanthoma elasticum
Objavljeno: 22.07.2019; Ogledov: 157; Prenosov: 0
.pdf Polno besedilo (689,32 KB)

2.
The Multifaceted Complexity of Genetic Diseases: A Lesson from Pseudoxanthoma Elasticum
Ivonne Pasquali Ronchetti, Giulia Annovi, Federica Boraldi, Daniela Quaglino, 2011, samostojni znanstveni sestavek ali poglavje v monografski publikaciji

Najdeno v: ključnih besedah
Povzetek najdenega: ...Genetic pathology, skin, morphology, PXE...
Ključne besede: Genetic pathology, skin, morphology, PXE
Objavljeno: 23.08.2019; Ogledov: 166; Prenosov: 0
.pdf Polno besedilo (23,60 MB)

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