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THE sphingolipidoses comprise a group of inherited metabolic diseases characterized by the accumulation of excessive quantities of certain glycolipids or phospholipids in various tissues of the body. These lipids have a common basic structure called ceramide (Fig. 1, line 1) consisting of the long-chain amino alcohol sphingosine acylated with a fatty acid on the nitrogen atom of sphingosine.Recent significant advances have provided considerable insight into the nature of the metabolic aberrations in several of the sphingolipidystrophies. This progress is due to a large extent to the application of newly developed technics for the chemical synthesis of specifically labeled sphingolipids. . . .
Roscoe O. Brady (Thu,) studied this question.