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Correction of the defective extracellular matrix of Ehlers‐Danlos syndrome skin fibroblasts by dexamethasone.
Author(s) -
Moro Laura,
Colombi Marina,
Zoppi Nicoletta,
Ghinelli Annalisa,
Barlati Sergio
Publication year - 1994
Publication title -
cell biology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.932
H-Index - 77
eISSN - 1095-8355
pISSN - 1065-6995
DOI - 10.1006/cbir.1994.1004
Subject(s) - fibronectin , extracellular matrix , dexamethasone , collagen vi , ehlers–danlos syndrome , messenger rna , cytoplasm , fibroblast , microbiology and biotechnology , matrix (chemical analysis) , western blot , extracellular , chemistry , biology , cell culture , medicine , pathology , endocrinology , biochemistry , gene , genetics , chromatography
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.