Glycosphingolipids catabolism

An inborn error of glycosphingolipid catabolism characterized by the accumulation of a ceramide derivative in blood and many tissues. It is due to a deficiency of one of the catabolic enzymes, a-D-galactoside galactohydrolase. It is transmitted by an X-linked gene. 

This part of the article discusses the in vitro catabolism of sugar chains in glycosphingolipids by mammalian exoglycosidases, with the foregoing points kept under consideration. 

In contrast to glycosphingolipids containing 2-acetamido-2-deoxy-D-galactose, virtually nothing is known about the catabolism of glycosphingolipids containing 2-acetamido-2-deoxy-D-glucose. 

The concept of the catabolism of glycosphingolipids by glycohydro-lases has entered a new era with the discovery of protein activators, but there are many questions remaining to be answered. What is the physiological role of the activators What is the mode of their action ... 

An article by Li and Li (Tnlane University, LA) on the biosynthesis and catabolism of glycosphingolipids serves to extend that by Kiss (Vol. 24), which dealt mainly with the chemistry of these compounds. Schwarz and Datema (Giessen) provide a detailed account of the lipid pathway of protein glyeosylation and of its inhibitors, and then discuss the biological significance of protein-bound carbohydrates, thereby... 

Figure 20-11 Biosynthesis and catabolism of glycosphingolipids. The heavy bars indicate metabolic blocks in known diseases.

Glycosphingolipids Function as Structural Components and as Specific Cell Receptors Defects in Sphingolipid Catabolism Are Associated with Metabolic Diseases... 

Catabolism of some glycosphingolipids. The glycosyl hydrolase enzymes involved in these reactions are localized in the lysosomes. 

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