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Sandhoff's disease is characterised by the accumulation of N-acetylhexosamine-containing oligosaccharides, both within glycosphingolipids and as free oligosaccharides, both of which contain N-acetyl moieties.
Structure of seven oligosaccharides excreted in the urine of a patient with Sandhoff's disease (GM2 gangliosidosis-variant O).