Ceramide glucose

The greatest advances at the enzyme level have been in understanding the inborn errors associated with lipid metabolism (27). At least nine of these diseases have been described at the enzyme level (Table II). The first five diseases listed in Table II involve enzymes which successively degrade Cer-Glc-Gal-(NeuNAc)-GalNAc-Gal to ceramide, glucose, galactose, and neuraminic acid. No genetic diseases associated... 

Basu, S., Kaufman, B. Roseman, S. (1968) Enzymatic Synthesis of Ceramide-glucose and Ceramide Lactose by Glycosyltransferases from Embryonic Chicken Brain , Journal of Biological Chemistry, 243, 5802-7... 

Chapter 3 Inborn Errors of Metabolism Ceramide Glucose Galactose Galactose N-acetylgalactosamine... 

SVENNEEHOLM (1962,1963), Klenk et al. (1963), Kuhn and Wiegandt (1963), Kunau (1964), and Klenk and Kunau (1964). They showed that it consists of one mol each of ceramide, glucose, galactose, N-acetyl-galactosamine and NANA according to the following formula (see fig. 4). 

There are several studies supporting the latter possibility. Brady s (1963) proposal that an enzymatic defect resulted in impairment of cleavage of the ceramide-glucose bond of the cerebrosides, was supported by studies on cerebrosides... 

It is probable that the stored lipid of ACD occurs normally in small amounts. SvENNERHOLM and SvENNERHOLM (1963) found a trihexoside with a proportion of ceramide glucose galactose of 1 1 2 in plasma and Martens son et al. (1966) found a trihexoside in kidney lipids of normals in addition to dihexoside. 

Tay-Sachs ganglioside Ceramide- Glucose (4—1) Galactose 1 3 I 1 NANA (4-1) xV-Acetyl galactosamine... 

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