Liégeois N J, Horner J W, DePinho R A
Department of Microbiology, Albert Einstein College of Medicine, Bronx, NY 10461, USA.
Proc Natl Acad Sci U S A. 1996 Feb 6;93(3):1303-7. doi: 10.1073/pnas.93.3.1303.
A genetic approach has been established that combines the advantages of blastocyst complementation with the experimental attributes of the developing lens for the functional analysis of genes governing cellular proliferation, terminal differentiation, and apoptosis. This lens complementation system (LCS) makes use of a mutant mouse strain, aphakia (ak), homozygotes of which fail to develop an ocular lens. We demonstrate that microinjection of wild-type embryonic stem (ES) cells into ak/ak blastocysts produces chimeras with normal ES-cell-derived lenses and that microinjection of Rb-/- ES cells generates an aberrant lens phenotype identical to that obtained through conventional gene targeting methodology. Our determination that a cell autonomous defect underlies the aphakia condition assures that lenses generated through LCS are necessarily ES-cell-derived. LCS provides for the rapid phenotypic analysis of loss-of-function mutations, circumvents the need for germ-line transmission of null alleles, and, most significantly, facilitates the study of essential genes whose inactivation is associated with early lethal phenotypes.
已建立一种遗传学方法,该方法结合了囊胚互补的优势与发育中的晶状体的实验特性,用于对控制细胞增殖、终末分化和细胞凋亡的基因进行功能分析。这种晶状体互补系统(LCS)利用了一种突变小鼠品系——无晶状体(ak),其纯合子无法发育出眼晶状体。我们证明,将野生型胚胎干细胞(ES细胞)显微注射到ak/ak囊胚中可产生具有正常ES细胞来源晶状体的嵌合体,而将Rb-/- ES细胞显微注射则会产生与通过传统基因靶向方法获得的异常晶状体表型相同的表型。我们确定无晶状体状况的基础是细胞自主性缺陷,这确保了通过LCS产生的晶状体必然是ES细胞来源的。LCS为功能丧失突变的快速表型分析提供了条件,避免了无效等位基因的种系传递需求,最重要的是,便于研究其失活与早期致死表型相关的必需基因。