title: Dermal fibroblasts from pseudoxanthoma elasticum patients have raised MMP-2 degradative potential.
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.
authors.affiliation: Department of Biomedical Sciences, University of Modena and Reggio Emilia, Via Campi 287, 41100 Modena, Italy. quaglino.daniela@unimore.it
authors.forename: Daniela
authors.initials: D
authors.lastname: Quaglino
authors.forename: Luigi
authors.initials: L
authors.lastname: Sartor
authors.forename: Spiridione
authors.initials: S
authors.lastname: Garbisa
authors.forename: Federica
authors.initials: F
authors.lastname: Boraldi
authors.forename: Antonietta
authors.initials: A
authors.lastname: Croce
authors.forename: Alberto
authors.initials: A
authors.lastname: Passi
authors.forename: Giancarlo
authors.initials: G
authors.lastname: De Luca
authors.forename: Roberta
authors.initials: R
authors.lastname: Tiozzo
authors.forename: Ivonne
authors.initials: I
authors.lastname: Pasquali-Ronchetti
mesh_terms: D000328:Adult; D001706:Biopsy; D002457:Cell Extracts / Q000032:analysis; D002478:Cells, Cultured; D003470:Culture Media / Q000737:chemistry; D005109:Extracellular Matrix / Q000201:enzymology / Q000378:metabolism; D005347:Fibroblasts / Q000201:enzymology*; D006801:Humans; D020778:Matrix Metalloproteinase 2 / Q000378:metabolism*; D008875:Middle Aged; D011561:Pseudoxanthoma Elasticum / Q000378:metabolism / Q000473:pathology*; D012333:RNA, Messenger / Q000378:metabolism; D012867:Skin / Q000473:pathology*
chemical_list: D002457:Cell Extracts; D003470:Culture Media; D012333:RNA, Messenger; D020778:Matrix Metalloproteinase 2