Correction of the DNA repair defect in Fanconi anemia complementation groups A and D cells
Correction of the DNA repair defect in Fanconi anemia complementation groups A and D cells
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DOI：
10.1006/bbrc.1996.6008
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发表时间：
1997-01-23
影响因子：
3.1
通讯作者：
Parrish, DD
中科院分区：
文献类型：
--
作者：
Lambert, MW;Tsongalis, GJ;Parrish, DD
我们以前已经从范可尼贫血，互补组A（FA-A）和D（FA-D）细胞中分离出一种DNA核酸内切酶复合物，该复合物在切割含有链间交联的DNA的能力上有缺陷，所述链间交联由peptide加WA光产生。FA复合物的修复能力，与相应的正常复合物相比，现在已经检查使用两种类型的互补分析。首先，通过电穿孔将正常复合物引入8-甲氧补骨脂素（8-MOP）加UVA处理的FA-A和FA-D细胞中，导致其修复缺陷得到纠正，这是通过测量修复相关的非程序性DNA合成（UDS）来确定的。FA-A和FA-D复合物可以类似地补充彼此细胞中的修复缺陷，但不能补充自己细胞中的修复缺陷。第二，在无细胞系统中混合正常与FA-A和FAD复合物，或FA-A与FA-D复合物，导致这些FA复合物切割受损DNA能力的缺陷得到纠正。这些结果表明，正常复合物含有校正FA-A和FA-D细胞中DNA修复缺陷所需的蛋白质，并且FA-A和FA-D复合物含有相互补充修复缺陷所需的蛋白质。(C)1997学术出版社
We have previously isolated from Fanconi anemia, complementation groups A (FA-A) and D (FA-D) cells, a DNA endonuclease complex which is defective in its ability to incise DNA containing interstrand crosslinks produced by psoralen plus WA light. The repair capabilities of the FA complexes, compared with those of the corresponding normal complex, have now been examined using two types of complementation analysis. First, introduction of the normal complex, by electroporation, into 8-methoxypsoralen (8-MOP) plus UVA treated FA-A and FA-D cells resulted in correction of their repair defect, determined by measuring repair-related unscheduled DNA synthesis (UDS), The FA-A and FA-D complexes could similarly complement the repair defect in each others' cells, but not in their own. Second, mixing the normal with the FA-A and FAD complexes, or the FA-A with the FA-D complex, in a cell-free system resulted in correction of the defect in ability of these FA complexes to incise damaged DNA. These results indicate that the normal complex contains the proteins needed to correct the DNA repair defect in FA-A and FA-D cells and that the FA-A and FA-D complexes contain the protein needed to complement the repair defect in each other. (C) 1997 Academic Press