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Glycosphingolipids in Health and Disease

Address correspondence to Frederick L. Kiechle, M.D., Ph.D., Department of Clinical Pathology, William Beaumont Hospital, 3601 West Thirteen Mile Road, Royal Oak, MI 48073-6769, USA; tel 248 551 8020; fax 248 551 3694; e-mail fkiechle{at}beaumont.edu.

Glycosphingolipids are ubiquitous membrane constituents that are subdivided in neutral or acidic fractions (gangliosides and sulfatides). Their analysis requires extraction and separation by thin-layer chromatography or high-performance liquid chromatography. Ganglioside composition changes occur in response to variations in cellular morphology and function. Glycosphingolipids are implicated in the pathogenesis of various diseases, including glycosphingolipidoses, peripheral neuropathies caused by anti-ganglioside antibodies, and secretory diarrhea. Gangliosides play a role in the induction of apoptosis. For example, ceramide-induced apoptosis is associated with increased synthesis of a ganglioside, GD3. Gangliosides are also potential diagnostic markers and therapeutic targets for cancer.

Keywords: Glycosphingolipids, gangliosides, sulfatides, glycosphingolipidosis, Tay-Sachs disease, Sandhoff disease, peripheral neuropathies, bacterial toxin receptor, apoptosis

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