ALTERATION OF MATRIX MACROMOLECULE SYNTHESIS BY FIBROBLASTS FROM A PATIENT WITH PACHYDERMOPERIOSTOSIS

Citation
Y. Wegrowski et al., ALTERATION OF MATRIX MACROMOLECULE SYNTHESIS BY FIBROBLASTS FROM A PATIENT WITH PACHYDERMOPERIOSTOSIS, Journal of investigative dermatology, 106(1), 1996, pp. 70-74
Citations number
34
Categorie Soggetti
Dermatology & Venereal Diseases
ISSN journal
0022202X
Volume
106
Issue
1
Year of publication
1996
Pages
70 - 74
Database
ISI
SICI code
0022-202X(1996)106:1<70:AOMMSB>2.0.ZU;2-A
Abstract
Pachydermoperiostosis (primary hypertrophic osteoarthropathy) is a ver y rare genetic disease characterized by pachydermia, periostosis, arth ralgia, and finger clubbing. Its pathophysiology is still unclear, but previous studies have reported connective tissue hypertrophy in the s kin of these patients. We investigated the synthesis of collagen, fibr onectin, and proteoglycans by fibroblasts from affected and unaffected skin from one patient with pachydermoperiostosis and four normal dono rs. We found that collagen synthesis was largely decreased in fibrobla sts from the diseased skin, whereas the synthesis of the small dermata n-sulfate-containing proteoglycan decorin strongly increased. Fibrobla sts from the unaffected skin of the patient exhibited syntheses of the se macromolecules similar to control fibroblasts from healthy donors. Northern blot and dot blot analyses showed decreased pro alpha(1)(I) c ollagen in patient's affected and unaffected skin fibroblasts whereas increased decorin mRNA levels were found in fibroblasts from the patie nt's affected skin. No change in cell proliferation was observed. Thes e data demonstrate an alteration of fibroblast biosynthetic activity i n the skin lesions of pachydermoperiostosis, which may be responsible, at least in part, for the patient's phenotype.