Abstract
Summary Three structurally related carbohydrate-rich fractions which are not present in normal urine have been isolated from the urine of GM1-gangliosidosis patients, Types 1 and 2. Their carbohydrate structures have been determined by permethylation studies and p.m.r. spectroscopy and shown to be identical to compounds isolated previously from the liver in this disease. The main compound has the structure [(Ga1 β1→4 G1cNAc β1→2)2 (Gal β1→-4 GlcNAc β1→4)] (Man α1→6, Man α1→3) Man β1→4 GlcNAc. These compounds appear to derive from the incomplete degradation of certain erythrocyte glycoproteins and plasma immunoglobulins.
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