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髓鞘相关糖蛋白的发育和病理生理学方面

Developmental and pathophysiological aspects of the myelin-associated glycoprotein.

作者信息

Brady R O, Quarles R H

机构信息

Developmental and Metabolic Neurology Branch, National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, Maryland 20892.

出版信息

Cell Mol Neurobiol. 1988 Jun;8(2):139-48. doi: 10.1007/BF00711240.

Abstract
  1. A glycoslylated sulfate-containing protein known as myelin-associated glycoprotein (MAG) appears to be unique to the central and peripheral nervous systems. This component has been characterized and cDNA clones have been isolated. 2. MAG is a member of the immunoglobulin superfamily. The principal form of MAG synthesized in brain during active myelination has an apparent molecular weight of 100,000. Alternate exon splicing leads to an additional 5000-dalton smaller form with a different C terminus. 3. In patients with multiple sclerosis, MAG is rapidly lost in areas of active disease. It is immunologically reactive in patients with benign monoclonal gammopathy associated with peripheral neuropathy. 4. The role of MAG in the formation of the myelin sheath and its participation in autoimmune neurological disorders are outlined.
摘要
  1. 一种名为髓鞘相关糖蛋白(MAG)的含硫酸化糖基化蛋白似乎是中枢神经系统和周围神经系统所特有的。该成分已得到表征,并且已分离出cDNA克隆。2. MAG是免疫球蛋白超家族的成员。在活跃髓鞘形成过程中大脑中合成的MAG的主要形式的表观分子量为100,000。可变外显子剪接导致形成一种额外的、C末端不同的小5000道尔顿的形式。3. 在多发性硬化症患者中,MAG在活跃病变区域迅速丢失。它在与周围神经病变相关的良性单克隆丙种球蛋白病患者中具有免疫反应性。4. 概述了MAG在髓鞘形成中的作用及其参与自身免疫性神经疾病的情况。

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