Repozitorij Univerze v Novi Gorici

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Naslov:Fibroblast protein profile analysis highlights the role of oxidative stress and vitamin K recycling in the pathogenesis of pseudoxanthoma elasticum.
Avtorji:Boraldi, Federica (Avtor)
Annovi, Giulia (Avtor)
Guerra, Deanna (Avtor)
Chiara, Paolinelli Devincenzi (Avtor)
Maria Immaculada, Garcia Fernandez (Avtor)
Panico, Fulvio (Avtor)
De Santis, Giorgio (Avtor)
Tiozzo, Roberta (Avtor)
Pasquali Ronchetti, Ivonne (Avtor)
Quaglino, Daniela (Avtor)
Datoteke:Gradivo nima datotek. Gradivo je morda fizično dosegljivo v knjižnici fakultete, zalogo lahko preverite v COBISS-u. Povezava se odpre v novem oknu
Jezik:Angleški jezik
Vrsta gradiva:Delo ni kategorizirano (r6)
Tipologija:1.01 - Izvirni znanstveni članek
Organizacija:UNG - Univerza v Novi Gorici
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
Leto izida:2009
Št. strani:1084-1098
Številčenje:3
COBISS_ID:5440507  Povezava se odpre v novem oknu
URN:URN:SI:UNG:REP:AJAK4E7V
DOI:10.1002/prca.200900007 Povezava se odpre v novem oknu
Licenca:CC BY-NC-ND 4.0
To delo je dosegljivo pod licenco Creative Commons Priznanje avtorstva-Nekomercialno-Brez predelav 4.0 Mednarodna
Število ogledov:76
Število prenosov:0
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Področja:Gradivo ni uvrščeno v področja.
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Skupna ocena:(0 glasov)
Vaša ocena:Ocenjevanje je dovoljeno samo prijavljenim uporabnikom.

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Gradivo je del revije

Naslov:Proteomics Clinical Apllication
Leto izida:2009

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