Differential expression of matrix metalloproteinases and proteoglycans in Juvenile Hyaline Fibromatosis

• Published in: Journal of dermatological science Vol. 61; no. 2; pp. 94 - 100
• Main Authors: Tzellos, Thrasivoulos G, Batzios, Spyros P, Dionyssopoulos, Alexander, Karakiulakis, George, Papakonstantinou, Eleni
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ireland Ltd 01.02.2011
• Subjects: Aggrecans - genetics; Aggrecans - metabolism; Biglycan - genetics; Biglycan - metabolism; Child; Decorin - genetics; Decorin - metabolism; Dermatology; Down-Regulation - genetics; Enzyme Precursors - metabolism; Extracellular Matrix; Female; Gelatinases; Gelatinases - metabolism; Gene Expression; Heparan Sulfate Proteoglycans - genetics; Heparan Sulfate Proteoglycans - metabolism; Humans; Hyaline Fibromatosis Syndrome - enzymology; Hyaline Fibromatosis Syndrome - genetics; Hyaline Fibromatosis Syndrome - metabolism; Juvenile Hyaline Fibromatosis; Matrix Metalloproteinase 2 - genetics; Matrix Metalloproteinase 2 - metabolism; Matrix Metalloproteinase 9 - genetics; Matrix Metalloproteinase 9 - metabolism; Matrix metalloproteinases; Proteoglycans; Skin - enzymology; Skin - metabolism; Statistics, Nonparametric; Tissue Inhibitor of Metalloproteinase-1 - metabolism; Tissue Inhibitor of Metalloproteinase-2 - metabolism; Tissue inhibitors of matrix metalloproteinases; Up-Regulation - genetics; Extracellular matrix; Matrix metalloproteinases; Proteoglycans; Gelatinases; Tissue inhibitors of matrix metalloproteinases; Juvenile Hyaline Fibromatosis

Abstract Background Juvenile Hyaline Fibromatosis (JHF) is a rare autosomal recessive disorder, histologically characterized by the production and deposition of an unidentified hyaline material in the skin and other organs. Extracellular matrix molecules are implicated in the development of skin les...