Deficiency of a membrane skeletal protein, 4.1G, results in myelin abnormalities in the peripheral nervous system.
Deficiency of a membrane skeletal protein, 4.1G, results in myelin abnormalities in the peripheral nervous system.
复制标题
膜骨骼蛋白 4.1G 的缺乏会导致周围神经系统中的髓磷脂异常。
DOI：
10.1007/s00418-017-1600-6
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发表时间：
2017
影响因子：
2.3
通讯作者：
Terada N.
中科院分区：
文献类型：
--
作者：
Saitoh Y;Ohno N;Yamauchi J;Sakamoto T;Terada N.
我们之前证明了一种膜骨架分子复合物，4.1 g -膜棕榈酰化蛋白6 (MPP6) -细胞粘附分子4，被纳入周围神经系统（PNS）的雪旺细胞。在这项研究中，我们评估了4.1 g缺陷（−/−）小鼠的运动活性和髓鞘超微结构。当被尾巴悬吊时，老龄4.1G−/−小鼠表现出痉挛性腿部伸展，特别是在过度劳累后。4.1G−/−小鼠的运动传导速度比野生型小鼠慢。电镜下，4.1G−/−小鼠表现出髓磷脂异常：髓磷脂在淋巴结间变厚，一些偏狂的髓磷脂尖端附着扭曲。此外，由于4.1G−/−坐骨神经中免疫定位的消失和Lin7c和Lin7a的产生减少，以及MPP6和Lin7与免疫沉淀的相互作用，我们发现了4.1G对支架蛋白Lin7分选的新功能。因此，我们在此提出4.1G作为PNS中髓磷脂正确形成的信号。
We previously demonstrated that a membrane skeletal molecular complex, 4.1G–membrane palmitoylated protein 6 (MPP6)–cell adhesion molecule 4, is incorporated in Schwann cells in the peripheral nervous system (PNS). In this study, we evaluated motor activity and myelin ultrastructures in 4.1G-deficient (−/−) mice. When suspended by the tail, aged 4.1G−/−mice displayed spastic leg extension, especially after overwork. Motor-conduction velocity in 4.1G−/−mice was slower than that in wild-type mice. Using electron microscopy, 4.1G−/−mice exhibited myelin abnormalities: myelin was thicker in internodes, and attachment of myelin tips was distorted in some paranodes. In addition, we found a novel function of 4.1G for sorting a scaffold protein, Lin7, due to disappearance of the immunolocalization and reduction of the production of Lin7c and Lin7a in 4.1G−/−sciatic nerves, as well as the interaction of MPP6 and Lin7 with immunoprecipitation. Thus, we herein propose 4.1G functions as a signal for proper formation of myelin in PNS.