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首页> 外文期刊>Glia >Proteolipid Protein Cannot Replace P-0 Protein as the Major Structural Protein of Peripheral Nervous System Myelin
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Proteolipid Protein Cannot Replace P-0 Protein as the Major Structural Protein of Peripheral Nervous System Myelin

机译:蛋白脂蛋白不能取代P-0蛋白作为周围神经系统髓磷脂的主要结构蛋白

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摘要

The central nervous system (CNS) of terrestrial vertebrates underwent a prominent molecular change when proteolipid protein (PLP) replaced P-0 protein as the most abundant protein of CNS myelin. However, PLP did not replace P-0 in peripheral nervous system (PNS) myelin. To investigate the possible consequences of a PLP to P-0 shift in PNS myelin, we engineered mice to express PLP instead of P-0 in PNS myelin (PLP-PNS mice). PLP-PNS mice had severe neurological disabilities and died between 3 and 6 months of age. Schwann cells in sciatic nerves from PLP-PNS mice sorted axons into one-to-one relationships but failed to form myelin internodes. Mice with equal amounts of P-0 and PLP had normal PNS myelination and lifespans similar to wild-type (WT) mice. When PLP was overexpressed with one copy of the P-0 gene, sciatic nerves were hypomyelinated; mice displayed motor deficits, but had normal lifespans. These data support the hypothesis that while PLP can co-exist with P-0 in PNS myelin, PLP cannot replace P-0 as the major structural protein of PNS myelin. GLIA 2015;63:66-77
机译:当蛋白脂蛋白(PLP)取代P-0蛋白成为CNS髓磷脂的最丰富蛋白时,陆生脊椎动物的中枢神经系统(CNS)发生了显着的分子变化。但是,PLP不能替代外周神经系统(PNS)髓磷脂中的P-0。为了调查PNS髓磷脂中PLP转变为P-0的可能后果,我们设计了小鼠在PNS髓磷脂中表达PLP而不是P-0(PLP-PNS小鼠)。 PLP-PNS小鼠患有严重的神经功能障碍,在3至6个月大时死亡。 PLP-PNS小鼠坐骨神经中的雪旺细胞将轴突分成一对一的关系,但未能形成髓鞘节。 P-0和PLP含量相等的小鼠具有正常的PNS髓鞘形成和寿命,类似于野生型(WT)小鼠。当PLP随P-0基因的一个拷贝过表达时,坐骨神经的髓鞘减少。小鼠表现出运动缺陷,但寿命正常。这些数据支持以下假设:虽然PLP可以与P-0在PNS髓磷脂中共存,但PLP不能取代P-0作为PNS髓磷脂的主要结构蛋白。 GLIA 2015; 63:66-77

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