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The Neuroscientist
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Pathology of Glycosphingolipid Metabolism: The Molecular and Cellular Basis of Neurodegenerative Disease

Michaela Wendeler

Kekulé-Institut für Organische Chemie und Biochemie, Universität Bonn, Bonn, Germany

Thomas Kolter

Kekulé-Institut für Organische Chemie und Biochemie, Universität Bonn, Bonn, Germany

Konrad Sandhoff

Kekulé-Institut für Organische Chemie und Biochemie, Universität Bonn, Bonn, Germany

Glycosphingolipids are ubiquitous constituents of eukaryotic plasma membranes. Genetically determined deficiencies in their catabolic pathways cause the excessive intralysosomal accumulation of these lipids and give rise to a group of inherited metabolic diseases, the sphingolipidoses. The progression of these disorders often involves severe degeneration of the nervous system, and for nearly all of them, no effective treatment is available to date. Here, we discuss the physiological functions of glycosphingolipids and the topology and mechanism of their metabolism. The molecular defects associated with these storage disorders as well as their pathophysiological consequences and potential therapeutic prospects are presented. Finally, the importance of recently available animal models for the investigation of pathogenesis and the evaluation of future therapy approaches is discussed.

Key Words: Glycosphingolipids • Lysosomal storage diseases • Sphingolipidoses • Sphingolipid activator proteins

The Neuroscientist, Vol. 6, No. 4, 285-296 (2000)
DOI: 10.1177/107385840000600411
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