globosidek
Globosidek, known in English as globosides, are a family of neutral glycosphingolipids found in the plasma membranes of animal cells. They consist of a ceramide lipid linked to an oligosaccharide chain that lacks sialic acid. The sugar chain is usually composed of several hexose residues, including galactose and N-acetylgalactosamine, arranged in distinct globoside series.
Members of the globoside family include globotriaosylceramide (Gb3) and globotetraosylceramide (Gb4), among others. Gb3 is the
Biosynthesis occurs in the Golgi apparatus, starting from ceramide. A series of glycosyltransferases adds sugars to
Clinical relevance: Gb3 accumulation due to deficiency of alpha-galactosidase A, as seen in Fabry disease, leads
See also: glycosphingolipids, globoside family, Gb3, Shiga toxin receptor, Fabry disease.