Smithies O, Kim H S
Department of Pathology, University of North Carolina, Chapel Hill 27599-7525.
Proc Natl Acad Sci U S A. 1994 Apr 26;91(9):3612-5. doi: 10.1073/pnas.91.9.3612.
Experimental analysis of complex quantitative genetic traits, such as essential hypertension, should be greatly facilitated by being able to manipulate the expression of a gene in living animals without altering the nucleotide sequence, chromosomal location, or regulatory elements of the gene. To explore this possibility, we have used targeted gene disruption and duplication to generate mice that are genetically identical [(129 x C57BL6)F1] except for having one, two, or three functional copies of the gene coding for angiotensinogen. The two-copy animals have two normal copies of the angiotensinogen gene; the one-copy and three-copy animals have one normal copy with the other either disrupted or duplicated by gene targeting. The duplicated pair of genes was generated by a special form of gap-repair gene targeting that tandemly duplicates the whole of a gene together with 5' and 3' flanking regions. We find progressively and significantly higher levels of the gene product in the animals having increasing numbers of gene copies: the one-copy animals have steady-state plasma angiotensinogen levels approximately 35% of normal (P < 0.0001), and the three-copy animals have levels approximately 124% of normal (P < 0.004). Detailed information about regulatory sequences is not required for this type of experiment; nor is it necessary to have DNA clones or targeting constructs that cover the whole of the target gene. Varying gene copy numbers by targeting consequently offers a promising approach to quantitative genetics.
对于诸如原发性高血压等复杂数量性状的实验分析,如果能够在不改变基因的核苷酸序列、染色体位置或调控元件的情况下,在活体动物中操纵基因表达,将极大地促进这一过程。为了探索这种可能性,我们利用靶向基因敲除和复制技术,培育出了除编码血管紧张素原的基因分别有一个、两个或三个功能拷贝外,其他基因完全相同的[(129xC57BL6)F1]小鼠。两个拷贝的动物有两个正常的血管紧张素原基因拷贝;一个拷贝和三个拷贝的动物有一个正常拷贝,另一个通过基因靶向技术被敲除或复制。通过一种特殊形式的缺口修复基因靶向技术产生了一对串联重复的基因,该技术将整个基因及其5'和3'侧翼区域一起串联重复。我们发现,基因拷贝数增加的动物体内基因产物水平逐步显著升高:一个拷贝的动物稳态血浆血管紧张素原水平约为正常水平的35%(P<0.0001),三个拷贝的动物水平约为正常水平的124%(P<0.004)。这类实验不需要关于调控序列的详细信息;也不需要有覆盖整个靶基因的DNA克隆或靶向构建体。因此,通过靶向技术改变基因拷贝数为数量遗传学提供了一种很有前景的方法。