AI Article Synopsis

  • The study examined cultured fibroblasts from normal subjects and those with Pseudoxanthoma elasticum (PXE) to assess the activity and expression of certain metalloproteases (MMPs) and their inhibitors.
  • The findings revealed that specific MMPs and TIMPs were not detected in either group, but MMP-2 levels were significantly higher in PXE fibroblasts compared to controls.
  • The results indicate that PXE fibroblasts exhibit increased potential for tissue breakdown, suggesting that MMP-2 might play a role in the connective tissue changes associated with this genetic condition.

Article Abstract

Cultured fibroblasts from the dermis of normal subjects and of Pseudoxanthoma elasticum (PXE) patients were analysed for enzyme activity, protein and mRNA expression of metalloproteases (MMP-2, MMP-3, MMP-9, MT1-MMP) and of their specific inhibitors (TIMP-1, TIMP-2 and TIMP-3). MMP-3, MMP-9 and TIMP-3 mRNAs and proteins failed to be detected in both the medium and the cell layer of both controls and PXE patients. MMP-2 mRNA was significantly more expressed in PXE than in control cell lines, whereas MT1-MMP, TIMP-1 and TIMP-2 mRNAs appeared unchanged. MMP-2 was significantly higher in the cell extracts from PXE fibroblasts than in control cells, whereas differences were negligible in the cell medium. Data suggest that PXE fibroblasts have an increased proteolytic potential, and that MMP-2 may actively contribute to connective tissue alterations in this genetic disorder.

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http://dx.doi.org/10.1016/j.bbadis.2004.09.012DOI Listing

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