Systemic sclerosis sera affect fibrillin-1 deposition by dermal blood microvascular endothelial cells: therapeutic implications of cyclophosphamide

INTRODUCTION: Systemic sclerosis (SSc) is a connective tissue disorder characterized by endothelial cell injury, autoimmunity and fibrosis. The following three fibrillin-1 alterations have been reported in SSc. (1) Fibrillin-1 microfibrils are disorganized in SSc dermis. (2) Fibrillin-1 microfibrils...

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Main Authors: Villano, Marilisa, Borghini, Annalisa, Manetti, Mirko, Gabbrielli, Erica, Rossi, Antonella, Sestini, Piersante, Milia, Anna Franca, Nacci, Francesca, Guiducci, Serena, Matucci-Cerinic, Marco, Ibba-Manneschi, Lidia, Weber, Elisabetta
Format: Artigo
Jezik:Inglês
Izdano: BioMed Central 2013
Teme:
Research Article
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC3978697/
https://ncbi.nlm.nih.gov/pubmed/23962393
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/ar4270
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