Akgün E, Zahn J, Baumes S, Brown G, Liang F, Romanienko P J, Lewis S, Jasin M
Cell Biology and Genetics Program, Sloan-Kettering Institute and Cornell University Graduate School of Medical Sciences, New York, New York 10021, USA.
Mol Cell Biol. 1997 Sep;17(9):5559-70. doi: 10.1128/MCB.17.9.5559.
Genetic instability is promoted by unusual sequence arrangements and DNA structures. Hairpin DNA structures can form from palindromes and from triplet repeats, and they are also intermediates in V(D)J recombination. We have measured the genetic stability of a large palindrome which has the potential to form a one-stranded hairpin or a two-stranded cruciform structure and have analyzed recombinants at the molecular level. A palindrome of 15.3 kb introduced as a transgene was found to be transmitted at a normal Mendelian ratio in mice, in striking contrast to the profound instability of large palindromes in prokaryotic systems. In a significant number of progeny mice, however, the palindromic transgene is rearranged; between 15 and 56% of progeny contain rearrangements. Rearrangements within the palindromic repeat occur both by illegitimate and homologous, reciprocal recombination. Gene conversion within the transgene locus, as quantitated by a novel sperm fluorescence assay, is also elevated. Illegitimate events often take the form of an asymmetric deletion that eliminates the central symmetry of the palindrome. Such asymmetric transgene deletions, including those that maintain one complete half of the palindromic repeat, are stabilized so that they cannot undergo further illegitimate rearrangements, and they also exhibit reduced levels of gene conversion. By contrast, transgene rearrangements that maintain the central symmetry continue to be unstable. Based on the observed events, we propose that one mechanism promoting the instability of the palindrome may involve breaks generated at the hairpin structure by a hairpin-nicking activity, as previously detected in somatic cells. Because mammalian cells are capable of efficiently repairing chromosome breaks through nonhomologous processes, the resealing of such breaks introduces a stabilizing asymmetry at the center of the palindrome. We propose that the ability of mammalian cells to eliminate the perfect symmetry in a palindromic sequence may be an important DNA repair pathway, with implications regarding the metabolism of palindromic repeats, the mutability of quasipalindromic triplet repeats, and the early steps in gene amplification events.
异常的序列排列和DNA结构会促进基因不稳定。发夹DNA结构可由回文序列和三联体重复序列形成,它们也是V(D)J重组的中间体。我们测量了一个大型回文序列的遗传稳定性,该回文序列有可能形成单链发夹或双链十字形结构,并在分子水平上分析了重组体。作为转基因引入的15.3 kb回文序列在小鼠中以正常孟德尔比率传递,这与原核系统中大型回文序列的高度不稳定性形成鲜明对比。然而,在大量子代小鼠中,回文转基因发生了重排;15%至56%的子代含有重排。回文重复序列内的重排通过非同源和同源、相互重组发生。通过一种新型精子荧光测定法定量的转基因位点内的基因转换也有所增加。非同源事件通常采取不对称缺失的形式,消除回文序列的中心对称性。这种不对称转基因缺失,包括那些保留回文重复序列完整一半的缺失,变得稳定,因此不能再进行进一步的非同源重排,并且它们的基因转换水平也降低。相比之下,保持中心对称性的转基因重排仍然不稳定。基于观察到的事件,我们提出促进回文序列不稳定的一种机制可能涉及先前在体细胞中检测到的由发夹切割活性在发夹结构处产生的断裂。由于哺乳动物细胞能够通过非同源过程有效修复染色体断裂,这种断裂的重新封闭在回文序列中心引入了一种稳定的不对称性。我们提出哺乳动物细胞消除回文序列中完美对称性的能力可能是一种重要的DNA修复途径,这与回文重复序列的代谢、准回文三联体重复序列的可变性以及基因扩增事件的早期步骤有关。