PURIFICATION AND STRUCTURAL-ANALYSIS OF EXTRACELLULAR-MATRIX OF A SKIN TUMOR FROM A PATIENT WITH JUVENILE HYALINE FIBROMATOSIS

Juvenile hyaline fibromatosis is a rare mesenchymal dysplasia that is inherited in an autosomal recessive fashion. The histological features of the tumor-like lesions are characterized by the deposition of amor phous hyaline material in the extracellular spaces of the dermis and s oft tissues. We have analyzed the hyaline substance in a specimen of a skin tumor obtained from a 4-year-old Japanese girl with juvenile hya line fibromatosis. It was found to consist mainly of type VI collagen; a small amount of type I collagen was also present. These components were separated by DEAE-cellulose ion-exchange chromatography under red ucing conditions. The ratio of the dry weights of type I and type VI c ollagen was 1:4. Of the three chains of type VI collagen (alpha 1(VI), alpha 2(VI) and alpha 3(VI)), alpha 3(VI) was the most abundant. Glyc osaminoglycans in the tumor tissue comprised dermatan sulfate, chondro itin sulfate and hyaluronan, with dermatan sulfate predominating. In c ontrast, hyaluronan is the most abundant in normal skin.