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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
Ključne besede: calcification, dermal fibroblast, elastic fiber, human skin, MGP, pseudoxanthoma elasticum
Objavljeno: 22.07.2019; Ogledov: 205; Prenosov: 0
.pdf Polno besedilo (689,32 KB)

2.
Fibroblast protein profile analysis highlights the role of oxidative stress and vitamin K recycling in the pathogenesis of pseudoxanthoma elasticum.
Ivonne Pasquali Ronchetti, Roberta Tiozzo, Giorgio De Santis, Fulvio Panico, Garcia Fernandez Maria Immaculada, Paolinelli Devincenzi Chiara, Deanna Guerra, Giulia Annovi, Federica Boraldi, Daniela Quaglino, 2009, izvirni znanstveni članek

Opis: Pseudoxanthoma elasticum (PXE) is a genetic disorder associated to mutations in the ABCC6 gene; however, the pathogenetic mechanisms leading to elastic fibre calcifications and to clinical manifestations are still unknown. Dermal fibroblasts, directly involved in the production of the extracellular milieu, have been isolated from healthy subjects and from patients affected by PXE, cultured in vitro and characterized for their ability to produce reactive oxygen species, for structural and functional properties of their cell membranes, for changes in their protein profile. Data demonstrate that oxidative stress has profound and endurable consequences on PXE fibroblast phenotype being responsible for: reduced levels of global DNA methylation, increased amount of carbonylated proteins and of lipid peroxidation products, altered structural properties of cell membranes, modified protein expression. Data shed new light on the pathogenetic pathways in PXE, by identifying a network of proteins affecting elastic fibre calcification through inefficient vitamin K recycling, and highlight the role of differentially expressed proteins as targets for validating the efficacy of future therapeutic strategies aiming to delay and/or revert the pathologic phenotype of PXE fibroblasts. Moreover, data open new perspectives for investigating PXE-like phenotypes in the absence of ABCC6 mutations.
Najdeno v: ključnih besedah
Povzetek najdenega: ...the pathogenetic mechanisms leading to elastic fibre calcifications and to clinical manifestations are still unknown....
Ključne besede: Ectopic calcification / Elastin / Fibroblast proteome / MRP6 / PXE
Objavljeno: 23.08.2019; Ogledov: 154; Prenosov: 0
.pdf Polno besedilo (650,81 KB)

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Fibroblasts from patients affected by Pseudoxanthoma elasticum exhibit an altered PPi metabolism and are more responsive to pro-calcifying stimuli
Daniela Quaglino, Angelica Bartolomeo, Giulia Annovi, Federica Boraldi, 2014, izvirni znanstveni članek

Najdeno v: ključnih besedah
Povzetek najdenega: ...Ectopic calcification Fibroblasts Pyrophosphate Pseudoxanthoma elasticum...
Ključne besede: Ectopic calcification Fibroblasts Pyrophosphate Pseudoxanthoma elasticum
Objavljeno: 23.08.2019; Ogledov: 162; Prenosov: 0
.pdf Polno besedilo (1,91 MB)

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