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Title:Fibroblast protein profile analysis highlights the role of oxidative stress and vitamin K recycling in the pathogenesis of pseudoxanthoma elasticum.
Authors:ID Boraldi, Federica, Università di Modena e Reggio Emilia (Author)
ID Annovi, Giulia, Università di Modena e Reggio Emilia (Author)
ID Guerra, Deanna, Università di Modena e Reggio Emilia (Author)
ID Chiara, Paolinelli Devincenzi, Università di Modena e Reggio Emilia (Author)
ID Maria Immaculada, Garcia Fernandez, University of Malaga (Author)
ID Panico, Fulvio, Università di Modena e Reggio Emilia (Author)
ID De Santis, Giorgio, Università di Modena e Reggio Emilia (Author)
ID Tiozzo, Roberta, Università di Modena e Reggio Emilia (Author)
ID Pasquali Ronchetti, Ivonne, Università di Modena e Reggio Emilia (Author)
ID Quaglino, Daniela, Università di Modena e Reggio Emilia (Author)
Files: This document has no files that are freely available to the public. This document may have a physical copy in the library of the organization, check the status via COBISS. Link is opened in a new window
Language:English
Work type:Not categorized
Typology:1.01 - Original Scientific Article
Organization:UNG - University of Nova Gorica
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
Year of publishing:2009
Number of pages:1084-1098
Numbering:3
PID:20.500.12556/RUNG-4694-22f46708-b67d-13a9-7253-f254d713abae New window
COBISS.SI-ID:5440507 New window
DOI:10.1002/prca.200900007 New window
NUK URN:URN:SI:UNG:REP:AJAK4E7V
Publication date in RUNG:23.08.2019
Views:4090
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Record is a part of a journal

Title:Proteomics Clinical Apllication
Year of publishing:2009

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License:CC BY-NC-ND 4.0, Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International
Link:http://creativecommons.org/licenses/by-nc-nd/4.0/
Description:The most restrictive Creative Commons license. This only allows people to download and share the work for no commercial gain and for no other purposes.
Licensing start date:22.08.2019

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