Myelin abnormalities in the optic and sciatic nerves in mice with GM1-gangliosidosis.
Myelin abnormalities in the optic and sciatic nerves in mice with GM1-gangliosidosis.
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DOI：
10.1177/1759091415568913
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发表时间：
2015-01
期刊：
影响因子：
4.7
通讯作者：
Seyfried TN
中科院分区：
文献类型：
--
作者：
Heinecke KA;Luoma A;d'Azzo A;Kirschner DA;Seyfried TN
GM1-神经节苷脂贮积症是一种鞘糖脂溶酶体贮积病，主要涉及 GM1 及其去唾液酸形式 (GA1) 的积累，主要在大脑中。使用薄层色谱和 X 射线衍射分析 7 个月和 10 个月大的 β-半乳糖苷酶 (β-gal) +/? 的脂质含量/组成以及视神经和坐骨神经的髓磷脂结构。和 β-gal −/− 小鼠，GM1 神经节苷脂沉积症模型。 β-gal -/− 小鼠的视神经重量低于未受影响的 β-gal +/? 小鼠的视神经重量。小鼠，但坐骨神经重量没有差异。 β-gal -/− 小鼠的视神经和坐骨神经中 GM1 和 GA1 的水平均显着增加。 β-gal -/− 小鼠视神经中富含髓磷脂的脑苷脂、硫苷脂和缩醛磷脂乙醇胺的含量显着低于 β-gal +/? 小鼠视神经中的含量。老鼠；然而，β-gal −/− 小鼠体内胆固醇酯含量丰富。在 β-gal -/− 小鼠的坐骨神经中没有检测到这些脂质的重大异常。 β-gal -/− 小鼠视神经中 GM1 和髓磷脂脂质的异常与髓磷脂相对量和新鲜神经周期性的减少相关。相比之下，对照小鼠和β-gal -/− 小鼠的坐骨神经中髓磷脂的相对量和周期性是无法区分的，这表明坐骨神经的病理学参与程度最小。我们的结果表明，在β-gal -/− 小鼠的视神经中观察到的神经化学病理学比在坐骨神经中更严重，这可能是由于视神经中糖脂储存量更大。
GM1-gangliosidosis is a glycosphingolipid lysosomal storage disease involving accumulation of GM1 and its asialo form (GA1) primarily in the brain. Thin-layer chromatography and X-ray diffraction were used to analyze the lipid content/composition and the myelin structure of the optic and sciatic nerves from 7- and 10-month old β-galactosidase (β-gal) +/? and β-gal −/− mice, a model of GM1gangliosidosis. Optic nerve weight was lower in the β-gal −/− mice than in unaffected β-gal +/? mice, but no difference was seen in sciatic nerve weight. The levels of GM1 and GA1 were significantly increased in both the optic nerve and sciatic nerve of the β-gal −/− mice. The content of myelin-enriched cerebrosides, sulfatides, and plasmalogen ethanolamines was significantly lower in optic nerve of β-gal −/− mice than in β-gal +/? mice; however, cholesteryl esters were enriched in the β-gal −/− mice. No major abnormalities in these lipids were detected in the sciatic nerve of the β-gal −/− mice. The abnormalities in GM1 and myelin lipids in optic nerve of β-gal −/− mice correlated with a reduction in the relative amount of myelin and periodicity in fresh nerve. By contrast, the relative amount of myelin and periodicity in the sciatic nerves from control and β-gal −/− mice were indistinguishable, suggesting minimal pathological involvement in sciatic nerve. Our results indicate that the greater neurochemical pathology observed in the optic nerve than in the sciatic nerve of β-gal −/− mice is likely due to the greater glycolipid storage in optic nerve.
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