Dermal fibroblasts from pseudoxanthoma elasticum patients have raised MMP-2 degradative potential
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...
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| Published in: | Biochimica et biophysica acta Vol. 1741; no. 1; pp. 42 - 47 |
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Elsevier B.V
30-06-2005
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| 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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| AbstractList | 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. |
| Author | Pasquali-Ronchetti, Ivonne Sartor, Luigi Garbisa, Spiridione Passi, Alberto Quaglino, Daniela Tiozzo, Roberta Croce, Antonietta Boraldi, Federica De Luca, Giancarlo |
| Author_xml | – sequence: 1 givenname: Daniela surname: Quaglino fullname: Quaglino, Daniela email: quaglino.daniela@unimore.it organization: Department of Biomedical Sciences, University of Modena and Reggio Emilia, Via Campi 287, 41100, Modena, Italy – sequence: 2 givenname: Luigi surname: Sartor fullname: Sartor, Luigi organization: Department of Experimental Biomedical Sciences, Medical School, University of Padova, Padova, Italy – sequence: 3 givenname: Spiridione surname: Garbisa fullname: Garbisa, Spiridione organization: Department of Experimental Biomedical Sciences, Medical School, University of Padova, Padova, Italy – sequence: 4 givenname: Federica surname: Boraldi fullname: Boraldi, Federica organization: Department of Biomedical Sciences, University of Modena and Reggio Emilia, Via Campi 287, 41100, Modena, Italy – sequence: 5 givenname: Antonietta surname: Croce fullname: Croce, Antonietta organization: Department of Biomedical Sciences, University of Modena and Reggio Emilia, Via Campi 287, 41100, Modena, Italy – sequence: 6 givenname: Alberto surname: Passi fullname: Passi, Alberto organization: Department of Experimental and Clinical Biomedical Sciences, University of Insubria, Varese, Italy – sequence: 7 givenname: Giancarlo surname: De Luca fullname: De Luca, Giancarlo organization: Department of Experimental and Clinical Biomedical Sciences, University of Insubria, Varese, Italy – sequence: 8 givenname: Roberta surname: Tiozzo fullname: Tiozzo, Roberta organization: Department of Biomedical Sciences, University of Modena and Reggio Emilia, Via Campi 287, 41100, Modena, Italy – sequence: 9 givenname: Ivonne surname: Pasquali-Ronchetti fullname: Pasquali-Ronchetti, Ivonne organization: Department of Biomedical Sciences, University of Modena and Reggio Emilia, Via Campi 287, 41100, Modena, Italy |
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| Keywords | Skin fibroblast Matrix degradation Pseudoxanthoma elasticum MMP-2 TIMP |
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| SubjectTerms | 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 |
| Title | Dermal fibroblasts from pseudoxanthoma elasticum patients have raised MMP-2 degradative potential |
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