STRUCTURE OF SULFATED MUCOPOLYSACCHARIDES FROM NORMAL TISSUES AND FROM PATIENTS WITH MUCOPOLYSACCHARIDOSES

Abstract

A comparative study on the structure of heparin, heparitin sulfates, and chondroitin sulfates from normal tissues and from patients with the Hunter and Hurler syndromes is described. The structural studies were based, among other methods, on the products formed from these compounds by the action of heparinase, heparitinases, chondroitinases, and other enzymes involved in the sequential degradation of these mucopolysaccharides in Flavobacterium heparinum. It is concluded that heparitin sulfate excreted by patients with the Hunter syndrome is a unique and complex structure composed of nine different types of disaccharide units and containing regions akin to heparitin sulfate from normal tissues and regions akin to heparin. It is also concluded that Hurler chondroitin sulfate B is a unique structure which is made almost exclusively of glycosidic linkages susceptible to chondroitinase B, whereas chondroitin sulfate B from normal tissues contain two regions: one susceptible to chondroitinase B and a dodecasaccharide region which is susceptible to chondroitinase AC. The implications of these findings on the possible metabolic errors of the Hunter and Hurler syndromes is discussed.