A study is reported of the brain glycoproteins and the glycosaminoglycans in a case of infantile metachromatic leucodystrophy, associated with a ponto-cerebellar systemic atrophy, verified by autopsy. The glycosaminoglycan concentrations in the grey matter were within the normal range, while in the white matter they were slightly increased. The glycopeptides from the soluble and insoluble glycoproteins of white matter were altered not only in their quantity but also in their composition. In the grey matter a decrease of all sugars except the NaNa was detected in the insoluble fraction while in the soluble no change was detected, except for a threefold increase of fucose. The relationship was discussed of soluble to insoluble glycoproteins, and a mechanism for their derangement in MLD was suggested.
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