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, 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.
Found in: osebi
Keywords: Ectopic calcification / Elastin / Fibroblast proteome / MRP6 / PXE
Published: 23.08.2019; Views: 2453; Downloads: 0
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2. Ectopic calcification in β-thalassemia patients is associated with increased oxidative stress and lower MGP carboxylationPaolo Cianciulli, Cees Vermeer, Leon J. Schurgers, Giulia Annovi, Chiara Paolinelli DeVincenzi, Maria Immaculada Garcia Fernandez, Federica Boraldi, Ivonne Pasquali Ronchetti, Daniela Quaglino, 2013, original scientific article Found in: osebi
Keywords: Elastin
Oxidative stress
Matrix Gla protein
Ectopic calcification
Beta-thalassemia
Published: 23.08.2019; Views: 2304; Downloads: 0
Fulltext (1,30 MB) |
3. Quality Control of Purified Proteins to Improve Research Data ReproducibilityStephan Uebel, Ario de Marco, Nick Berrow, Mario Lebendiker, Maria Garcia-Alai, Stefan Knauer, Blanca Lopez-Mendez, Andrea Matagne, Annabel Parret, Kim Remans, Bertrand Raynal, 2019, published scientific conference contribution abstract (invited lecture) Found in: osebi
Keywords: protein quality, biophysical analysis, aggregation
Published: 15.01.2020; Views: 1813; Downloads: 0
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4. Quality control of purified proteins to improve data quality and reproducibility: results from a large-scale surveyArio De Marco, Bertrand Raynal, Kim Remans, Annabel Parret, Blanca Lopez-Mendez, André Matagne, Maria Garcia-Alai, Stefan H. Knauer, Mario Lebendiker, Stephan Uebel, Nick Berrow, 2021, original scientific article Found in: osebi
Keywords: protein quality, biophysical analysis, protein aggregates
Published: 22.04.2021; Views: 1291; Downloads: 0
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5. Quality control of protein reagents for the improvement of research data reproducibilityBertrand Raynal, Stephan Uebel, Kim Remans, Annabel Parret, André Matagne, Blanca Lopez-Mendez, Stefan H. Knauer, Maria Garcia-Alai, Mario Lebendiker, Nick Berrow, Ario De Marco, 2021, short scientific article Found in: osebi
Keywords: protein quality, protein aggregation, data reproducibility
Published: 17.05.2021; Views: 1275; Downloads: 8
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