Correction of the defective extracellular matrix of Ehlers-Danlos syndrome skin fibroblasts by dexamethasone.
Article first published online: 2 JAN 2013
© The Author(s) Journal compilation © 1994 International Federation for Cell Biology
Cell Biology International
Volume 18, Issue 1, pages 29–37, January 1994
How to Cite
Moro, L., Colombi, M., Zoppi, N., Ghinelli, A. and Barlati, S. (1994), Correction of the defective extracellular matrix of Ehlers-Danlos syndrome skin fibroblasts by dexamethasone. Cell Biology International, 18: 29–37. doi: 10.1006/cbir.1994.1004
- Issue published online: 2 JAN 2013
- Article first published online: 2 JAN 2013
- Paper received 26.10.93. Revised paper accepted 28.12.93.
- Cited By
Skin fibroblasts derived from Ehlers-Danlos syndrome (EDS) patients lack an organized extracellular matrix (ECM) of fibronectin (FN) and often show an accumulation of cytoplasmic FN. The treatment of EDS cells of different types (I to VIII) with 10-7 M dexamethasone (dex), as well as cocultivation with control fibroblasts, induced in most cases the assembly of a FN-like ECM. The study of FN mRNA expression by dot-blot hybridization and of FN released into the culture media of EDS cells showed that the correction of the defective FN-ECM of EDS cells by dex treatment is associated in most cases with an increase of FN mRNA synthesis and of secreted FN.