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人类颅神经的神经节苷脂组成,特别涉及米勒·费雪综合征的病理生理学

Ganglioside composition of the human cranial nerves, with special reference to pathophysiology of Miller Fisher syndrome.

作者信息

Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I

机构信息

Department of Neurology, Faculty of Medicine, University of Tokyo, Japan.

出版信息

Brain Res. 1997 Jan 16;745(1-2):32-6. doi: 10.1016/s0006-8993(96)01123-7.

DOI:10.1016/s0006-8993(96)01123-7
PMID:9037391
Abstract

Total ganglioside fractions from the human cranial nerves purified on a Phenyl Sepharose column, were given mild alkaline treatment, after which their composition and amounts of lipid-bound sialic acid were determined by HPTLC-densitometry with resorcinol as the coloring reagent. The total amounts of lipid-bound sialic acid were 156.5 ng/mg of wet tissue in the Ist cranial nerve (olfactory tract) and 131.9 ng/mg in the IInd nerve, greater than the amounts in the other nerves (99.1-120.0 ng/mg). The Ist, IInd, and VIIIth nerves had GM4, but not LM1. It may reflect their histological feature of the central nervous system. The IIIrd, IVth, and VIth nerves, as well as the IInd, had significantly higher percentages of GQ1b (11.6-13.2%) than the other nerves (5.2-8.4%). The high proportion of GQ1b specific to these three cranial nerves involved in the ocular movement lends support to the role of serum anti-GQ1b antibody in the pathogenetic mechanisms of ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome.

摘要

在苯基琼脂糖柱上纯化的人颅神经总神经节苷脂组分经过温和的碱性处理,之后用间苯二酚作为显色剂,通过高效薄层层析光密度法测定其脂质结合唾液酸的组成和含量。在第Ⅰ颅神经(嗅束)中脂质结合唾液酸的总量为156.5 ng/mg湿组织,在第Ⅱ颅神经中为131.9 ng/mg,高于其他神经中的含量(99.1 - 120.0 ng/mg)。第Ⅰ、Ⅱ和Ⅷ颅神经含有GM4,但不含LM1。这可能反映了它们作为中枢神经系统的组织学特征。第Ⅲ、Ⅳ和Ⅵ颅神经以及第Ⅱ颅神经中GQ1b的百分比(11.6 - 13.2%)显著高于其他神经(5.2 - 8.4%)。参与眼球运动的这三条颅神经中GQ1b的高比例支持了血清抗GQ1b抗体在米勒·费希尔综合征和格林 - 巴利综合征眼肌麻痹发病机制中的作用。

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