Ivonne Pasquali Ronchetti (Author), Federica Boraldi (Author), Giulia Annovi (Author), Paolo Cianciulli (Author), Federica Boraldi (Author), Daniela Quaglino (Author)

Abstract

Soft connective tissue calcification is not a passive process, but the consequence of metabolic changes of local mesenchymal cells that, depending on both genetic and environmental factors, alter the balance between pro- and anti-calcifying pathways. While the role of smooth muscle cells and pericytes in ectopic calcifications has been widely investigated, the involvement of fibroblasts is still elusive. Fibroblasts isolated from the dermis of pseudoxanthoma elasticum (PXE) patients and of patients exhibiting PXE-like clinical and histopathological findings offer an attractive model to investigate the mechanisms leading to the precipitation of mineral deposits within elastic fibers and to explore the influence of the genetic background and of the extracellular environment on fibroblast-associated calcifications, thus improving the knowledge on the role of mesenchymal cells on pathologic mineralization.

Keywords

fibroblasts;PXE;PXE-likedisorders;elastin;extracellularmatrix;ectopiccalcification;mesenchymal stromalcells;

Data

Language: English
Year of publishing:
Typology: 1.02 - Review Article
Organization: UNG - University of Nova Gorica
UDC: 61
COBISS: 5442043 Link will open in a new window
ISSN: 1664-8021
Views: 2470
Downloads: 0
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Other data

URN: URN:SI:UNG
Type (COBISS): Not categorized
Pages: str. 1-16
Issue: ǂVol. ǂ4
Chronology: Mar. 2013
DOI: 10.3389/fgene.2013.00022
ID: 11206428