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Therapy for the sphingolipidoses.

Abstract
Sphingolipidoses are human metabolic storage disorders characterized by the accumulation of harmful quantities of glycosphingolipids and phosphosphingolipids. These lipids have in common a hydrophobic portion of their structure called ceramide. In glycosphingolipids, various oligosaccharides are linked to ceramide through glycosidic bonds. An example is glucocerebroside, composed of ceramide and 1 molecule of glucose. Large quantities of glucocerebroside accumulate in tissues in patients with Gaucher disease. Higher oligosaccharide homologues contain additional neutral and acidic oligosaccharides. Among these are gangliosides that have 1 or more molecules of N-acetylneuraminic acid. A ganglioside called G(M2) accumulates in Tay-Sachs disease. Sphingomyelin is a phosphosphingolipid that accumulates in patients with Niemann-Pick disease.
AuthorsR O Brady
JournalArchives of neurology (Arch Neurol) Vol. 55 Issue 8 Pg. 1055-6 (Aug 1998) ISSN: 0003-9942 [Print] United States
PMID9708954 (Publication Type: Journal Article, Review)
Topics
  • Humans
  • Sphingolipidoses (enzymology, therapy)

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