CAJ | 학술논문

某些蛋白翻译水平的激活和抑制对胚胎发育过程中基因表达起到重要的时空调节作用.在果蝇中,Bicaudal-c(dBic-C)基因产物被认为是一种能与RNA结合的蛋白质分子,并对转录后水平具有重要的调节作用.dBic-C基因的突变会导致果蝇缺失头部结构和重复的尾部结构,故而命名为双尾-C基因.后来又在多个不同物种(如线虫、爪蟾、小鼠)中发现dBic-C的同源基因,并且这些同源基因的蛋白质产物高度保守.小鼠的Bicaudal-C基因(Biccl)定位于10号染色体的C1区域,其蛋白质N端含有KH结构域,用于介导蛋白质与RNA的相瓦作用;C端的SAM结构域则在蛋白质与蛋白质相互结合中发挥作用.目前已发现3种与Biccl基因相关的小鼠突变体模型jcpk、bpk和67Gso,这些小鼠突变体中Biccl基因在不同位点发生突变,产生异常的蛋白质产物,结果导致小鼠肾脏发生胞囊化,并且影响身体其他器官的正常形态及功能,病理表型特征与人类的多囊肾病(polycystic kidney disease,PKD)极为相似.本文通过总结和同顾Bicaudal-C基因研究的成果和进展,揭示该基因在生物体发育和器官形成中的作用,为深入研究这一基因的生物学功能奠定基础.
모사단백번역수평적격활화억제대배태발육과정중기인표체기도중요적시공조절작용.재과승중,Bicaudal-c(dBic-C)기인산물피인위시일충능여RNA결합적단백질분자,병대전록후수평구유중요적조절작용.dBic-C기인적돌변회도치과승결실두부결구화중복적미부결구,고이명명위쌍미-C기인.후래우재다개불동물충(여선충、조섬、소서)중발현dBic-C적동원기인,병차저사동원기인적단백질산물고도보수.소서적Bicaudal-C기인(Biccl)정위우10호염색체적C1구역,기단백질N단함유KH결구역,용우개도단백질여RNA적상와작용;C단적SAM결구역칙재단백질여단백질상호결합중발휘작용.목전이발현3충여Biccl기인상관적소서돌변체모형jcpk、bpk화67Gso,저사소서돌변체중Biccl기인재불동위점발생돌변,산생이상적단백질산물,결과도치소서신장발생포낭화,병차영향신체기타기관적정상형태급공능,병리표형특정여인류적다낭신병(polycystic kidney disease,PKD)겁위상사.본문통과총결화동고Bicaudal-C기인연구적성과화진전,게시해기인재생물체발육화기관형성중적작용,위심입연구저일기인적생물학공능전정기출.
Translational activation and repression play an important role in the spatial-temporal regulation of gene expression in embryonic development.Bicaudal-C is an RNA-binding molecule believed to function at this post-transcriptional level.Loss-of-function mutants in Drosophila affect anterior-posterior patterning.producing abnormal embryos lacking head formation and with duplicate posterior segments.The Xenopus homologue of Bicaudal-C is one of the few molecules.When microinjected ectopically,it results in endoderm formation in the absence of mesodelTa induction.The homologues of Bicaudal-C in many other species(C:elegans,Xenopus,mouse,human,erc.)are also found to be very conserved.Mouse Bic-C gene(Biccl)is localized to a C1 region located on Chromosome 10,and the Biccl gene has two alternatively spliced transcripts:transcript A and B.Bicaudal-C contains several conserved N-terminal KH domains and a conserved C-terminal SAM domain.The KH domains have been shown to bind RNA,and the SAM domain is thought to play a role in protein-protein interactions.Thore are three mouse mutant models for Biccl,jcpk,bpk,and 67Gso.Mutations in different sites of Biccl in these models produce the cystic phenotypes in the kidney and affect the normal formation/functions of other organs(liver,pancreas and erc.),which are very similar to human polycystic kidney disease(PKD).In this review,we outline the recent progress in Bicaudal-C research and appraise its functions in development and organ formation.