核糖体蛋白S26的研究进展

Research Progress of Ribosomal Protein S26

核糖体蛋白(ribosomal proteins,RPs)不仅在细胞内参与合成蛋白质,还具有多种核糖体外功能。核糖体蛋白S26(RPS26)位于核糖体小亚基,其功能障碍与多种疾病密切相关。近年来,有关RPS26的研究主要在参与核糖体装配等核糖体功能方面,以及参与无义介导的mRNA降解机制(nonsense-mediated mRNA decay,NMD)、直接或间接调控重要的抑癌基因p53表达等核糖体外功能方面。多篇报道证实RPS26基因突变可引起戴-布二氏贫血(Diamond-Blackfan anemia,DBA),而RPS26基因与Ⅰ型糖尿病的关系仍有争议。探索RPS26参与NMD机制在DBA发生中的作用有助于深入认识DBA发病机理,同时也可为完善SMaRT(spliceosome-mediated mRNA trans-splicing)技术等基因疗法提供帮助。此外,RPS26在癌症中的作用也值得进一步探索。

Ribosomal proteins （RPs） not only participate in the synthesis of proteins in cells, but also have a variety of extraribosomal functions. The ribosomal protein S26 （RPS26） locates in the small subunit of ribosome, and its dysfunction is closely related to many diseases. Recent researches on RPS26 include the ribosomal functions such as participating in ribosome assembly, and extraribosomal functions such as participating in the nonsense-mediated mRNA decay （NMD）, and directly or indirectly regulating the expression of the impor- tant tumor suppressor gene p53. Several reports confirmed that RPS26 gene mutations cause Diamond- Blackfan anemia （DBA）, and the relationship of RPS26 gene and type 1 diabetes （T1D） is still controversial. Exploring the role of RPS26 involved in NMD, which leads to DBA, helps to understand the pathogenesis of DBA, and also improve the efficiency of gene therapy with SMART （spliceosome-mediated RNA trans-splic- ing） method. In addition, the role of RPS26 in tumor formation is also worth exploring.