Biosynthesis and metabolic degradation of sphingolipids not containing sialic acid

Abstract

Interest in sphingolipid metabolism has increased rapidly during the past decade, and many of the steps involved in the biosynthesis and metabolic degradation of sphingolipids are now known. In this review these studies are critically exmined. Emphasis has been placed on the in vitro studies with cell-free systems, since these represent the groundwork for further purification and characterization of the enzyme systems involved. Experimental problems specific to this field of study, and the manner in which these may affect interpretation of experimental results, are discussed.

Keywords

• long-chain bases
• glycosphingolipids
• inborn errors of sphingolipid metabolism
• ceramide
• sphingomyelin
• cerebroside