Author： Bodo M. Baroni T. Carinci F. Becchetti E. Conte C. Bellucci C. Pezzetti F. Calvitti M. Bellocchio S. Stabellini G. Carinci P.
Publisher： Academic Press
ISSN： 1043-4666
Source： Cytokine, Vol.12, Iss.8, 2000-08, pp. : 1280-1283
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Abstract
The present study provides the first evidence that fibroblasts obtained from patients affected by Crouzon syndrome, a rare craniosynostosis, despite mutations in the high-affinity bFGF receptor retain their capacity to respond to bFGF. The growth factor reduces IL-1 secretion, downregulates biglycan and procollagen α1(I), and increases betaglycan expression. Since betaglycan is a co-receptor for bFGF signalling, an alternative signal transduction pathway is suggested in Crouzon fibroblasts, to explain the documented changes in ECM macromolecule production.
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