The sphiningolipidoses: an overview.

Abstract

An outline of the pathways of catabolism of four sphingolipids to ceramide, along with structural details of a few constituents, serves as a framework for better understanding of the sphingolipidoses. The four sphingolipids are sulfatide, sphingomyelin, globoside, and ganglioside GM1. Diseases which can be incorporated into the scheme include Niemann-Pick disease, Gaucher disease, metachromatic leukodystrophy, Krabbe disease, ceramide lactoside lipidosis, Tay-Sachs disease, generalized gangliosidosis, Fabry disease, and Sandhoff disease. Fucosidosis probably also belongs with this group. GM3 (hematoside) sphingolipodystrophy involves blocks in synthetic rather than catabolic pathways.

• Authors: H H West
• Journal: Postgraduate medicine (Postgrad Med) Vol. 61 Issue 3 Pg. 90-3, 95 (Mar 1977) ISSN: 0032-5481 [Print] England
• PMID: 402652 (Publication Type: Journal Article, Research Support, U.S. Gov't, Non-P.H.S.)

Chemical References

• Gangliosides
• Glycosphingolipids
• Sphingolipids
• Sphingomyelins
• Sulfoglycosphingolipids
• Galactosylgalactosylglucosylceramidase

Topics

• Galactosylgalactosylglucosylceramidase (metabolism)
• Gangliosides (metabolism)
• Glycosphingolipids (metabolism)
• Humans
• Sphingolipidoses (metabolism)
• Sphingolipids (metabolism)
• Sphingomyelins (metabolism)
• Sulfoglycosphingolipids (metabolism)