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Metabolite Card

Note

Galabiosylceramide is a non-acidic diglycosphingolipids, i.e. a sphingolipid with two or more carbohydrate moieties attached to a ceramide unit. It is a vital component of cellular membranes of most eukaryotic organisms and some bacteria. Its abundance relative to other lipids is usually low other than in epithelial and neuronal cells. Galabiosylceramide is one of the lipids that accumulate in excessive amounts in Fabry's disease. Fabry disease (FD) is an X-linked inborn error of glycosphingolipid (GSL) metabolism, caused by a deficiency of the lysosomal alpha-galactosidase A, which results in high levels in lysosomes and biological fluids of galabiosylceramide, also known as digalactosylceramide (Ga2). In Fabry disease, accumulation of galabiosylceramide is observed by coloration methods associated to optic or electron microscopy or more recently, with imaging mass spectrometry. Galabiosylceramide is the precursor of a series of oligoglycosylceramides. In animal tissues, biosynthesis involves addition of a second monosaccharide unit from the appropriate sugar nucleotide to a monoglycosylceramide, catalysed by a glycosyl transferase, in the lumen of the Golgi apparatus. Glycolipids are important components of the body's immune defense system, either in haptenic reactivity or in antibody-producing potency, i.e. as cellular immunogens or antigens. (PMID: 15702404, 15959771, 16324673, 14761135).