Dermal fibroblasts from pseudoxanthoma elasticum patients have raised MMP-2 degradative potential

• Published in: Biochimica et biophysica acta Vol. 1741; no. 1; pp. 42 - 47
• Main Authors: Quaglino, Daniela, Sartor, Luigi, Garbisa, Spiridione, Boraldi, Federica, Croce, Antonietta, Passi, Alberto, De Luca, Giancarlo, Tiozzo, Roberta, Pasquali-Ronchetti, Ivonne
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 30-06-2005
• Subjects: Adult; Biopsy; Cell Extracts - analysis; Cells, Cultured; Culture Media - chemistry; Extracellular Matrix - enzymology; Extracellular Matrix - metabolism; Fibroblasts - enzymology; Humans; Matrix degradation; Matrix Metalloproteinase 2 - metabolism; Middle Aged; MMP-2; Pseudoxanthoma elasticum; Pseudoxanthoma Elasticum - metabolism; Pseudoxanthoma Elasticum - pathology; RNA, Messenger - metabolism; Skin - pathology; Skin fibroblast; TIMP; Skin fibroblast; Matrix degradation; Pseudoxanthoma elasticum; MMP-2; TIMP
• ISSN: 0925-4439; 0006-3002; 1879-260X
• DOI: 10.1016/j.bbadis.2004.09.012

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.