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1.
Protein quality control for improved data reproducibility and reliability
Kim Remans, Ario De Marco, Nick Berrow, Mario Lebendiker, Maria Garcia-Alai, Stefan Knauer, Andre Matagne, Anne Perret, Stephan Uebel, Bertrand Raynal, 2022, published scientific conference contribution abstract

Keywords: protein quality, evaluation assays, biophysical methods
Published in RUNG: 13.04.2023; Views: 1608; Downloads: 0
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Quality Control of Purified Proteins to Improve Research Data Reproducibility
Stephan 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)

Keywords: protein quality, biophysical analysis, aggregation
Published in RUNG: 15.01.2020; Views: 3517; Downloads: 0
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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, 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.
Keywords: Ectopic calcification / Elastin / Fibroblast proteome / MRP6 / PXE
Published in RUNG: 23.08.2019; Views: 4237; Downloads: 0
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