Abstract

We are interested in the metabolic aberrations which occur in patients with sphingolipidoses and in particular in Gaucher's disease. We have recently synthesized some glucoselabeled cerebroside and have examined the catabolism of this compound in normal rat and human spleen tissues. We have found a very active enzyme which splits this material in these tissues and we are particularly interested in examining spleen tissues from Caucher's patients to try to determine if this particular enzyme is present or absent in tissues from these patients.

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