Biochemical characterization of scleroderma-inducing glycosaminoglycan.

The scleroderma-inducing N-sulfated glycosaminoglycan previously isolated by us from the urine of patients with systemic scleroderma was further purified: it was biochemically characterized by low O-sulfation and relative high N-sulfation. Consistent with this finding, desulfated and N-resulfated heparin, which had a similar composition to the urine-derived scleroderma-inducing glycosaminoglycan, induced a significant degree of sclerotic fibrosis in the skin of mice which had received intraperitoneal injections of it, whereas N-desulfated heparin with contrasting sulfation to it failed to cause any significant change in the skin.