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脱髓鞘疾病中的髓鞘相关糖蛋白

Myelin-associated glycoprotein in demyelinating disorders.

作者信息

Quarles R H

机构信息

Laboratory of Molecular and Cellular Neurobiology, National Institutes of Health, Bethesda, Maryland.

出版信息

Crit Rev Neurobiol. 1989;5(1):1-28.

PMID:2475262
Abstract

The myelin-associated glycoprotein (MAG) is a 100-kD, integral membrane glycoprotein that is a member of the immunoglobulin superfamily and appears to function in interactions between myelin-forming cells (both oligodendrocytes and Schwann cells) and the axolemma. Its early loss in the development of multiple sclerosis plaques in comparison to other myelin proteins suggests that it could play a key role in the pathogenesis of this disease. This selective loss of MAG may relate to the high susceptibility of human MAG to cleavage by a Ca2+-activated neutral protease. Human MAG also contains a highly immunogenic carbohydrate determinant that is also expressed on other neural glycoconjugates and is the antigen recognized by many human IgM paraproteins that occur in patients with peripheral neuropathy. This article emphasizes the role of MAG in multiple sclerosis and peripheral neuropathies occurring in association with IgM paraproteinemia.

摘要

髓鞘相关糖蛋白(MAG)是一种100kD的整合膜糖蛋白,属于免疫球蛋白超家族,似乎在形成髓鞘的细胞(少突胶质细胞和施万细胞)与轴膜之间的相互作用中发挥作用。与其他髓鞘蛋白相比,它在多发性硬化斑块形成过程中早期丢失,这表明它可能在该疾病的发病机制中起关键作用。MAG的这种选择性丢失可能与人类MAG对钙激活中性蛋白酶切割的高度敏感性有关。人类MAG还含有一种高度免疫原性的碳水化合物决定簇,该决定簇也存在于其他神经糖缀合物上,并且是许多外周神经病患者体内出现的人类IgM副蛋白所识别的抗原。本文强调了MAG在多发性硬化以及与IgM副蛋白血症相关的外周神经病中的作用。

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