Glycolipids — Intracellular Movement and Storage Diseases
Glycolipids — Intracellular Movement and Storage Diseases
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糖脂 — 细胞内运动和储存疾病
DOI：
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发表时间：
1990
期刊：
影响因子：
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通讯作者：
P. Leinekugel
中科院分区：
文献类型：
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作者：
K. Sandhoff;P. Leinekugel
鞘糖脂 (GSL) 是动物细胞质膜的成分。它们通过疏水性神经酰胺（N-酰基鞘氨醇）部分锚定在细胞膜上，而亲水性单糖或寡糖部分面向细胞外空间。鞘糖脂与糖蛋白和糖胺聚糖一起形成细胞表面的糖被。 GSL 模式是个体细胞类型、分化阶段和致癌转化的特征（Hakomori 1980；van Echten 和 Sandhoff，1989）。尽管一些含有 GSL 的唾液酸已被确定为毒素和病毒的结合位点（Yamakawa 和 Nagai，1978；Markwell 等，1981），但它们的生理功能仍然不清楚。到目前为止，大约100个GSL的结构已经被阐明。含有唾液酸的 GSL，称为神经节苷脂，是神经元表面的典型脂质，主要存在于灰质中（Lowden 和 Wolfe，1964；Derry 和 Wolfe，1967）。髓磷脂的主要成分硫脂和半乳糖神经酰胺在少突胶质细胞中占主导地位，而球系列的糖脂在成纤维细胞中占主导地位。 GSL代谢的任何紊乱将主要影响相应GSL集中的组织。
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.