1. 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, izvirni znanstveni članek Ključne besede: Elastin
Oxidative stress
Matrix Gla protein
Ectopic calcification
Beta-thalassemia
Objavljeno v RUNG: 23.08.2019; Ogledov: 4035; Prenosov: 0
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2. 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, 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.
Ključne besede: Ectopic calcification / Elastin / Fibroblast proteome / MRP6 / PXE
Objavljeno v RUNG: 23.08.2019; Ogledov: 4193; Prenosov: 0
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3. The effect of serum withdrawal on the protein profile of quiescent human dermal fibroblasts in primary cell culture.Boraldi Federica, Annovi Giulia, Paolinelli Devincenzi Chiara, Tiozzo Roberta, Quaglino Daniela, 2008, izvirni znanstveni članek Opis: The effect of serum deprivation on proliferating cells is well known, in contrast its role on primary
cell cultures, at confluence, has not been deeply investigated. Therefore, in order to explore
the response of quiescent cells to serum deprivation, ubiquitous mesenchymal cells, as normal
human dermal fibroblasts, were grown, for 48 h after confluence, in the presence or absence of
10% FBS. Fibroblast behaviour (i.e. cell morphology, cell viability, ROS production and elastin
synthesis) was evaluated morphologically and biochemically. Moreover, the protein profile was
investigated by 2-DE and differentially expressed proteins were identified by MS. Serum withdrawal
caused cell shrinkage but did not significantly modify the total cell number. ROS production,
as evaluated by the dihydroethidium (DH2) probe, was increased after serum deprivation,
whereas elastin synthesis, measured by a colorimetric method, was markedly reduced in the
absence of serum. By proteome analysis, 41 proteins appeared to significantly change their
expression, the great majority of protein changes were related to the cytoskeleton, the stress response
and the glycolytic pathway. Data indicate that human dermal fibroblasts in primary cell
culture can adapt themselves to environmental changes, without significantly altering cell viability,
at least after a few days of treatment, even though serum withdrawal represents a stress
condition capable to increase ROS production, to influence cell metabolism and to interfere with
cell behaviour, favouring the expression of several age-related features.
Ključne besede: Dermal fibroblasts / Primary cell culture / ROS production / Serum withdrawal
Objavljeno v RUNG: 22.07.2019; Ogledov: 4064; Prenosov: 0
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4. 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, 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.
Ključne besede: calcification, dermal fibroblast, elastic fiber, human skin, MGP, pseudoxanthoma elasticum
Objavljeno v RUNG: 22.07.2019; Ogledov: 4190; Prenosov: 0
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