Keeney S, Eker A P, Brody T, Vermeulen W, Bootsma D, Hoeijmakers J H, Linn S
Division of Biochemistry and Molecular Biology, University of California, Berkeley 94720.
Proc Natl Acad Sci U S A. 1994 Apr 26;91(9):4053-6. doi: 10.1073/pnas.91.9.4053.
Cells from a subset of patients with the DNA-repair-defective disease xeroderma pigmentosum complementation group E (XP-E) are known to lack a DNA damage-binding (DDB) activity. Purified human DDB protein was injected into XP-E cells to test whether the DNA-repair defect in these cells is caused by a defect in DDB activity. Injected DDB protein stimulated DNA repair to normal levels in those strains that lack the DDB activity but did not stimulate repair in cells from other xeroderma pigmentosum groups or in XP-E cells that contain the activity. These results provide direct evidence that defective DDB activity causes the repair defect in a subset of XP-E patients, which in turn establishes a role for this activity in nucleotide-excision repair in vivo.
已知患有DNA修复缺陷疾病色素性干皮病互补组E（XP - E）的部分患者的细胞缺乏DNA损伤结合（DDB）活性。将纯化的人DDB蛋白注入XP - E细胞，以测试这些细胞中的DNA修复缺陷是否由DDB活性缺陷引起。注入的DDB蛋白在那些缺乏DDB活性的菌株中可将DNA修复刺激到正常水平，但在来自其他色素性干皮病组的细胞或具有该活性的XP - E细胞中却不能刺激修复。这些结果提供了直接证据，证明DDB活性缺陷导致了一部分XP - E患者的修复缺陷，这反过来又确立了该活性在体内核苷酸切除修复中的作用。
Zotero 插件