Ziegler A, Leffell D J, Kunala S, Sharma H W, Gailani M, Simon J A, Halperin A J, Baden H P, Shapiro P E, Bale A E
Department of Therapeutic Radiology, Yale School of Medicine, New Haven, CT 06510.
Proc Natl Acad Sci U S A. 1993 May 1;90(9):4216-20. doi: 10.1073/pnas.90.9.4216.
To identify the sites in the p53 tumor suppressor gene most susceptible to carcinogenic mutation by sunlight, the entire coding region of 27 basal cell carcinomas (BCCs) of the skin was sequenced. Fifty-six percent of tumors contained mutations, and these were UV-like: primarily CC-->TT or C-->T changes at dipyrimidine sites. Such mutations can alter more than half of the 393 amino acids in p53, but two-thirds occurred at nine sites at which mutations were seen more than once in BCC or in 27 previously studied squamous cell carcinomas of the skin. Seven of these mutation hotspots were specific to skin cancers. Internal-cancer hotspots not located at dipyrimidine sites were not mutated in skin cancers; moreover, UV photoproducts were absent at these nucleotides. The existence of hotspots altered the process of inactivating p53 in BCC compared to other cancers: allelic loss was rare, but 45% of the point mutations were accompanied by a second point mutation on the other allele. At least one of each pair was located at a hotspot. Sunlight, acting at mutation hotspots, appears to cause mutations so frequently that it is often responsible for two genetic events in BCC development.
为了确定p53肿瘤抑制基因中最易受阳光致癌突变影响的位点,对27例皮肤基底细胞癌(BCC)的整个编码区进行了测序。56%的肿瘤含有突变,这些突变类似紫外线所致:主要是二嘧啶位点处的CC→TT或C→T变化。此类突变可改变p53中393个氨基酸的一半以上,但三分之二的突变发生在9个位点,这些位点在BCC或之前研究的27例皮肤鳞状细胞癌中不止一次出现突变。其中7个突变热点是皮肤癌特有的。位于二嘧啶位点以外的癌症内部热点在皮肤癌中未发生突变;此外,这些核苷酸处不存在紫外线光产物。与其他癌症相比,热点的存在改变了BCC中p53失活的过程:等位基因缺失很少见,但45%的点突变伴有另一个等位基因上的第二个点突变。每一对突变中至少有一个位于热点。阳光作用于突变热点,似乎如此频繁地引发突变,以至于它常常在BCC发展过程中导致两个遗传事件。