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Content, composition and molecular weight distribution of acidic glycosaminoglycans (GAGs) were determined in liver from five patients with genetic lysosomal storage diseases (Hurler syndrome, Hunter syndrome of severe type, Morquio syndrome, GM1-gangliosidosis type II and I-cell disease). There was a 30- to 40-fold increase in GAGs content of liver from patients with Hurler and Hunter syndromes. The GAGs accumulated in the livers consisted mainly of heparan sulfate and dermatan sulfate, and had a much lower molecular weight than those from control liver. The major GAG accumulated in liver from Morquio syndrome was keratan sulfate, which was not found in the livers from control and other patients, and chondroitin-6-sulfate was also increased. The content and the composition of liver GAGs from GM1-gangliosidosis and I-cell disease were similar to those of control liver. However, there was about a 33-fold increase in the amount of hexose on the liver GAGs from GM1-gangliosidosis. A molar ratio of sialic acid to hexosamine was 1.35 for the liver GAGs from I-cell disease and that of hexose to hexosamine was 8.47, while they were 0.46 and 2.32, respectively, for the control liver.

Acidic glycosaminoglycans in liver from five patients with mucopolysaccharidosis and mucolipidosis.