PMID: 8592084Jan 1, 1996Paper

Alteration of matrix macromolecule synthesis by fibroblasts from a patient with pachydermoperiostosis

The Journal of Investigative Dermatology
Y WegrowskiF X Maquart

Abstract

Pachydermoperiostosis (primary hypertrophic osteoarthropathy) is a very rare genetic disease characterized by pachydermia, periostosis, arthralgia, and finger clubbing. Its pathophysiology is still unclear, but previous studies have reported connective tissue hypertrophy in the skin of these patients. We investigated the synthesis of collagen, fibronectin, and proteoglycans by fibroblasts from affected and unaffected skin from one patient with pachydermoperiostosis and four normal donors. We found that collagen synthesis was largely decreased in fibroblasts from the diseased skin, whereas the synthesis of the small dermatan-sulfate-containing proteoglycan decorin strongly increased. Fibroblasts from the unaffected skin of the patient exhibited syntheses of these macromolecules similar to control fibroblasts from healthy donors. Northern blot and dot blot analyses showed decreased pro alpha 1 (I) collagen in patient's affected and unaffected skin fibroblasts whereas increased decorin mRNA levels were found in fibroblasts from the patient's affected skin. No change in cell proliferation was observed. These data demonstrate an alteration of fibroblast biosynthetic activity in the skin lesions of pachydermoperiostosis, which may be...Continue Reading

References

Feb 1, 1991·Journal of Structural Biology·R FleischmajerJ D Termine
Jun 1, 1988·The Biochemical Journal·J E Scott
Oct 1, 1986·Proceedings of the National Academy of Sciences of the United States of America·T Krusius, E Ruoslahti
Jan 1, 1987·Scandinavian Journal of Rheumatology·M Matucci-CerinicD Orlic
Nov 11, 1983·Journal of Chromatography·G BellonJ P Borel
Jan 1, 1995·The British Journal of Dermatology·L BianchiG Nini
Dec 1, 1994·Journal of the American Academy of Dermatology·A OikarinenM Kallioinen

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Citations

Jun 11, 2003·The Kaohsiung Journal of Medical Sciences·Wen-Chi YangWen-Chan Tsai
Nov 15, 2005·Clinical Genetics·M CastoriB Dallapiccola
Jan 25, 2005·International Journal of Dermatology·Serafinella P CannavòMario Vaccaro
Sep 13, 2002·American Journal of Physiology. Lung Cellular and Molecular Physiology·Narayanan VenkatesanMara S Ludwig
Jun 17, 1998·Matrix Biology : Journal of the International Society for Matrix Biology·A M HockingD J McQuillan

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