颤抖小鼠大脑中髓鞘相关糖蛋白的异常糖基化

Abnormal glycosylation of myelin-associated glycoprotein in quaking mouse brain.

作者信息

Konat G, Hogan E L, Leskawa K C, Gantt G, Singh I

机构信息

Department of Neurology, Medical University of South Carolina, 171 Ashley Ave., Charleston, SC 29425, U.S.A.

出版信息

Neurochem Int. 1987;10(4):555-8. doi: 10.1016/0197-0186(87)90084-2.

DOI:10.1016/0197-0186(87)90084-2

PMID:20501130

Abstract

Brain slices from actively myelinating (26-28 days) quaking and normal littermates were dual-labeled with radioactive mannose and fucose for 2 h. Following the incubation myelin was isolated by sucrose density gradient centrifugation and the incorporation of sugars into the major myelinassociated glycoprotein (MAG) determined. The incorporation of mannose (an internal monosaccharide) and fucose (a terminal monosaccharide) was impaired in quaking by approximately 70 and 83% respectively as compared to control. The mannose/fucose ratio in quaking myelin was approximately 70% higher than in control. The results indicate an abnormal processing of the N-linked oligosaccharide moiety of MAG in quaking oligodendrocytes.

摘要

取自正在进行髓鞘形成（26 - 28天）的颤抖小鼠和正常同窝小鼠的脑片，用放射性甘露糖和岩藻糖进行双标记2小时。孵育后，通过蔗糖密度梯度离心分离髓鞘，并测定糖类掺入主要髓鞘相关糖蛋白（MAG）的情况。与对照相比，颤抖小鼠中甘露糖（一种内部单糖）和岩藻糖（一种末端单糖）的掺入分别受损约70%和83%。颤抖小鼠髓鞘中的甘露糖/岩藻糖比值比对照高约70%。结果表明，在颤抖小鼠的少突胶质细胞中，MAG的N - 连接寡糖部分存在异常加工。