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轴周蛋白L-periaxin作为外周神经系统中特异表达的骨架蛋白之一,在髓鞘成熟与维护过程中发挥重要作用.肌营养不良相关蛋白2(DRP2)是目前报道与L-periaxin存在明确相互作用的唯一蛋白,但两蛋白精细的互作区域及互作的分子机制仍不清楚.本研究通过双分子荧光互补、免疫共沉淀、GST pull-down、荧光光谱、细胞定位等技术,进一步揭示了periaxin蛋白的核定位信号NLS结构域上3段不同的亚结构域中,NLS1只参与periaxin蛋白的核定位,NLS2和NLS3参与DRP2蛋白的互作;DRP2蛋白中spectrin like2和WW结构域参与和L-periaxin蛋白的互作.L-periaxin与DRP2互作区域的精细定位,为进一步在蛋白水平探讨L-periaxin与脱髓鞘型腓骨肌萎缩症4F亚型的发生机制之间的关系提供参考.
As one of the specific skeletal proteins expressed in the peripheral nervous system, L-periaxin plays an important role in the process of myelin maturation and maintenance.Dysorrhythmic-associated protein 2 (DRP2) is presently reported to interact with L-periaxin But the precise interaction between the two proteins and the molecular mechanism of the interaction remain unclear.In this study, we further revealed the interaction between bimolecular fluorescence and co-immunoprecipitation, GST pull-down, fluorescence spectrometry and cell localization NLS1 only participates in the nuclear localization of periaxin protein in NLS domain, and NLS2 and NLS3 are involved in the interaction of DRP2 protein; in DRP2 protein, both spectrin like2 and WW domain are involved and L-periaxin protein.The precise mapping of L-periaxin and DRP2 interaction region provides a reference for further discussing the relationship between L-periaxin and the pathogenesis of demyelinating Charfrial 4F subtype 4F at the protein level .