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腓骨肌萎缩症4F亚型是Periaxin基因的突变所导致一种脱髓鞘型遗传病.Periaxin蛋白是外周神经系统中特异且大量表达的蛋白,在髓鞘成熟与维护中发挥重要作用.而Ezrin是一种膜骨架连接蛋白,在细胞形态的维持、运动、黏附等方面发挥重要作用.在前期已证实L-periaxin与Ezrin间存在蛋白互作的基础上,本文通过分子荧光互补实验,结合免疫荧光定位实验、免疫共沉淀等技术,进一步分析并揭示了L-periaxin蛋白与Ezrin蛋白之间的互作方式,具体为L-periaxin(1-200 aa)与Ezrin(1-296 aa)以及L-periaxin(1060-1461 aa)与Ezrin(475-585 aa)以“头对头”与“尾对尾”的方式发生相互作用.Ezrin可能是一种引导L-periaxin在施万细胞膜上堆积的新的分子配体,二者可能通过蛋白分子间更加紧密的方式完成在细胞膜处的堆积,参与到髓鞘的维护中.
Charcot-Marie-Tooth Syndrome 4F subtype is a demyelinating genetic disease caused by mutation of Periaxin gene, which is a specific and heavily expressed protein in the peripheral nervous system and plays an important role in myelin maturation and maintenance. Ezrin Is a membrane scaffolding protein that plays an important role in the maintenance of cell morphology, movement, adhesion, etc. In the previous study, we confirmed the existence of protein interaction between L-periaxin and Ezrin. Based on the complementation of molecular fluorescence, Fluorescence localization experiment and co-immunoprecipitation technique were used to further analyze and reveal the interaction between L-periaxin and Ezrin protein. The specific interaction between L-periaxin (1-200 aa) and Ezrin (1-296 aa) and L -periaxin (1060-1461 aa) interacts with Ezrin (475-585 aa) in a “head to head” and “tail to tail.” Ezrin may be a pathway that directs L-periaxin on Schwann cell membranes On the accumulation of new molecular ligands, the two may be more closely through the protein molecules in the cell membrane accumulation, involved in the maintenance of myelin.