Proteoglycan alterations in skin fibroblast cultures from patients affected with pseudoxanthoma elasticum

A. Passi, R. Albertini, M. Baccarani Contri, G. De Luca, A. De Paepe, G. Pallavicini, I. Pasquali Ronchetti, R. Tiozzo

Research output: Contribution to journalArticlepeer-review

Abstract

Proteoglycans (PGs) were investigated in fibroblast cultures from both apparently normal and involved areas of skin from two patients affected with Pseudoxanthoma elasticum (PXE) and compared to control normal cells. Biochemical analysis showed that cells from the PXE-affected patients produced a PG population with stronger polyanion properties, as well as a markedly increased amount of high hydrodynamic-size PGs. Moreover, PGs from PXE-affected cells showed abnormal hydrophobic interaction properties when examined under associative conditions and included heparan sulphate (HS)-containing populations with anomalous electrophoretic mobility. These phenomena were particularly evident in the case of PGs secreted into the growth medium. In agreement with these findings immunohistochemical study showed alterations affecting decorin and biglycan, as well as a different content and distribution of HS-PGs in PXE-affected cells. The same biochemical and morphological alterations were confirmed for both patients on different cell cultures and were present in cells from both apparently normal and affected skin areas, being more pronounced in the latter. Our results indicate that PXE-affected fibroblasts in culture exhibit an abnormal PG metabolism, which could affect the normal assembly of extracellular matrix.

Original languageEnglish
Pages (from-to)111-120
Number of pages10
JournalCell Biochemistry and Function
Volume14
Issue number2
DOIs
Publication statusPublished - 1996

Keywords

  • Fibroblasts
  • Proteoglycans
  • Pseudoxanthoma elasticum
  • Skin

ASJC Scopus subject areas

  • Clinical Biochemistry
  • Cell Biology

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