Interleukin secretion, proteoglycan and procollagen alpha(1)(I) gene expression in Crouzon fibroblasts treated with basic fibroblast growth factor.
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 alpha(1)(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.
Bodo
M
M
Dipartimento di Medicina Sperimentale e Scienze Biochimiche, Università di Perugia, Italy. bodo@unipg.it
Baroni
T
T
Carinci
F
F
Becchetti
E
E
Conte
C
C
Bellucci
C
C
Pezzetti
F
F
Calvitti
M
M
Bellocchio
S
S
Stabellini
G
G
Carinci
P
P
D000293:Adolescent; D000328:Adult; D019898:Autocrine Communication; D003094:Collagen / Q000096:biosynthesis / Q000235:genetics*; D003394:Craniofacial Dysostosis / Q000378:metabolism* / Q000473:pathology; D004797:Enzyme-Linked Immunosorbent Assay; D016222:Fibroblast Growth Factor 2 / Q000378:metabolism / Q000502:physiology*; D005347:Fibroblasts / Q000378:metabolism*; D015870:Gene Expression; D006801:Humans; D007378:Interleukins / Q000378:metabolism*; D008562:Membrane Glycoproteins / Q000235:genetics / Q000378:metabolism; D011509:Proteoglycans / Q000096:biosynthesis / Q000235:genetics* / Q000378:metabolism; D012333:RNA, Messenger / Q000096:biosynthesis; D018125:Receptors, Transforming Growth Factor beta / Q000235:genetics / Q000378:metabolism; D053667:Syndecans
D007378:Interleukins; D008562:Membrane Glycoproteins; D011509:Proteoglycans; D012333:RNA, Messenger; D018125:Receptors, Transforming Growth Factor beta; D053667:Syndecans; D016222:Fibroblast Growth Factor 2; C071736:betaglycan; D003094:Collagen