Glycolipids — Intracellular Movement and Storage Diseases
Glycosphingolipids (GSL) are components of plasmamembranes of animal cells. They are anchored in the cellular membrane by their hydrophobic ceramide (N-acylsphingosine) moiety, while the hydrophilic mono- or oligosaccharide part faces the extracellular space. Together with glycoproteins and glycosaminoglycanes glycosphingolipids form the glycocalix of cell surfaces. The GSL-patterns are characteristic for individual cell types, stages of differentiation and oncogenic transformation (Hakomori 1980; van Echten and Sandhoff, 1989). Though some of the sialic acid containing GSL have been identified as binding sites for toxins and viruses (Yamakawa and Nagai, 1978; Markwell et al., 1981), their physiological functions remain obscure. The structure of about 100 GSL could be elucidated thus far. Sialic acid-containing GSL, called gangliosides, are typical lipids of neuronal surfaces and are predominant in the grey matter (Lowden and Wolfe, 1964; Derry and Wolfe, 1967). Sulfatide and galactosylceramide as main components of myelin prevail on oligodendrocytes while glycolipids of the globoseries predominate on fibroblasts. Any disorder in metabolism of GSL would mainly affect those tissues in which the correspondent GSL is concentrated.
KeywordsSialic Acid Culture Skin Fibroblast Golgi Vesicle Metachromatic Leukodystrophy Lipid Storage Disorder
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