High production of type VI collagen in multiple fibromatosis with multiple articular dysplasia

A patient with multiple fibromatosis occurring at the sites of multiple cartilagenous dysplasia was described. Collagen types solubilized with pepsin from the fibromatous tissue were fractionated by a different salt concentration and analyzed by SDS-polyacrylamide gel electrophoresis, which indicated that the tissue produces predominantly "short-chain" collagen. Western blotting of the subunits indicated a cross reaction with antisera of the type VI collagen. The results of rotatory shadowing electron microscopy confirmed the characteristic short-chain structure.