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1.
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: ...Ectopic calcification / Elastin / Fibroblast proteome / MRP6 / PXE... ...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: 152; Prenosov: 0
.pdf Polno besedilo (650,81 KB)

2.
Matrix Gla Protein and Alkaline Phosphatase Are Differently Modulated in Human Dermal Fibroblasts from PXE Patients and Controls
Deanna Guerra, Roberta Tiozzo, Tommaso Trenti, Leon J. Schurgers, Cees Vermeer, Giulia Annovi, Federica Boraldi, Daniela Quaglino, 2013, izvirni znanstveni članek

Najdeno v: ključnih besedah
Povzetek najdenega: ...Fibroblast, Vitamin K, Elastin calcification,Proteomics...
Ključne besede: Fibroblast, Vitamin K, Elastin calcification, Proteomics
Objavljeno: 23.08.2019; Ogledov: 215; Prenosov: 0
.pdf Polno besedilo (3,86 MB)

3.
Ectopic calcification in β-thalassemia patients is associated with increased oxidative stress and lower MGP carboxylation
Paolo Cianciulli, Cees Vermeer, Leon J. Schurgers, Giulia Annovi, Chiara Paolinelli DeVincenzi, Maria Immaculada Garcia Fernandez, Federica Boraldi, Ivonne Pasquali Ronchetti, Daniela Quaglino, 2013, izvirni znanstveni članek

Najdeno v: ključnih besedah
Povzetek najdenega: ... Elastin Oxidative stress Matrix Gla protein Ectopic calcification Beta-thalassemia...
Ključne besede: Elastin Oxidative stress Matrix Gla protein Ectopic calcification Beta-thalassemia
Objavljeno: 23.08.2019; Ogledov: 227; Prenosov: 0
.pdf Polno besedilo (1,30 MB)

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