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Correction of the defective extracellular matrix of Ehlers-Danlos syndrome skin fibroblasts by dexamethasone.

✍ Scribed by Laura Moro; Marina Colombi; Nicoletta Zoppi; Annalisa Ghinelli; Sergio Barlati

Publisher
Elsevier Science
Year
1994
Tongue
English
Weight
770 KB
Volume
18
Category
Article
ISSN
1065-6995

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✦ Synopsis

Abstract

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.

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