1. Matrix Gla Protein and Alkaline Phosphatase Are Differently Modulated in Human Dermal Fibroblasts from PXE Patients and ControlsFederica Boraldi, Giulia Annovi, Cees Vermeer, Leon J. Schurgers, Tommaso Trenti, Roberta Tiozzo, Deanna Guerra, Daniela Quaglino, 2013, original scientific article Keywords: Fibroblast, Vitamin K, Elastin calcification, Proteomics Published in RUNG: 23.08.2019; Views: 4453; Downloads: 0 This document has many files! More... |
2. Ectopic calcification in β-thalassemia patients is associated with increased oxidative stress and lower MGP carboxylationFederica Boraldi, Maria Immaculada Garcia Fernandez, Chiara Paolinelli DeVincenzi, Giulia Annovi, Leon J. Schurgers, Cees Vermeer, Paolo Cianciulli, Ivonne Pasquali Ronchetti, Daniela Quaglino, 2013, original scientific article Keywords: Elastin
Oxidative stress
Matrix Gla protein
Ectopic calcification
Beta-thalassemia Published in RUNG: 23.08.2019; Views: 4069; Downloads: 0 This document has many files! More... |
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4. Fibroblast protein profile analysis highlights the role of oxidative stress and vitamin K recycling in the pathogenesis of pseudoxanthoma elasticum.Federica Boraldi, Giulia Annovi, Deanna Guerra, Paolinelli Devincenzi Chiara, Garcia Fernandez Maria Immaculada, Fulvio Panico, Giorgio De Santis, Roberta Tiozzo, Ivonne Pasquali Ronchetti, Daniela Quaglino, 2009, original scientific article Abstract: 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. Keywords: Ectopic calcification / Elastin / Fibroblast proteome / MRP6 / PXE Published in RUNG: 23.08.2019; Views: 4235; Downloads: 0 This document has many files! More... |
5. 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: 4222; Downloads: 0 This document has many files! More... |