Schutte M, Hruban R H, Geradts J, Maynard R, Hilgers W, Rabindran S K, Moskaluk C A, Hahn S A, Schwarte-Waldhoff I, Schmiegel W, Baylin S B, Kern S E, Herman J G
Department of Oncology, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21205, USA.
Cancer Res. 1997 Aug 1;57(15):3126-30.
The Rb/p16 tumor-suppressive pathway is abrogated frequently in human tumors, either through inactivation of the Rb or p16INK4a/CDKN2/MTS1 tumor-suppressor proteins, or through alteration or overexpression of the cyclin D1 or cyclin-dependent kinase 4 oncoproteins. We reported previously that the p16 gene was genetically inactivated in 82% of pancreatic carcinomas. Nearly half of these inactivations were by intragenic mutation of p16, and the remainder were by homozygous deletion of the gene. Here, we analyzed pancreatic carcinomas for additional mechanisms by which the Rb/p16 pathway might be inactivated. Transcriptional silencing of the p16 gene in association with methylation of its 5'-CpG island was examined by methylation-specific PCR in 18 pancreatic carcinomas. Nine of these were known to harbor an intragenic mutation in p16, and nine had a wild-type p16 coding sequence. Seven of the 18 tumors were hypermethylated, and all 7 were p16 wild-type (P = 0.001). Complete silencing of transcription from methylated wild-type gene sequences was demonstrated. Immunohistochemical analysis revealed normal expression levels of the Rb protein in all carcinomas studied. None of the carcinomas had genomic amplification of the cyclin D1 or CDK4 genes, and none had mutation of the p16-binding domain of CDK4. An additional p16 mutation was identified. In total, the Rb/p16 pathway was abrogated in 49 of the 50 carcinomas (98%) studied, all through inactivation of the p16 gene. Similar results were obtained in an independently analyzed series of 19 pancreatic carcinomas. These data demonstrate the central role of the Rb/p16 pathway in the development of pancreatic carcinoma.
在人类肿瘤中,Rb/p16肿瘤抑制通路常常被破坏,其方式包括Rb或p16INK4a/CDKN2/MTS1肿瘤抑制蛋白的失活,或细胞周期蛋白D1或细胞周期蛋白依赖性激酶4癌蛋白的改变或过表达。我们之前报道过,82%的胰腺癌中p16基因发生了基因失活。这些失活中近一半是由于p16基因内突变,其余则是由于该基因的纯合缺失。在这里,我们分析了胰腺癌中Rb/p16通路可能被失活的其他机制。通过甲基化特异性PCR检测了18例胰腺癌中与p16基因5'-CpG岛甲基化相关的转录沉默情况。其中9例已知存在p16基因内突变,9例具有野生型p16编码序列。18例肿瘤中有7例发生了高甲基化,且这7例均为p16野生型(P = 0.001)。已证实甲基化的野生型基因序列的转录完全沉默。免疫组织化学分析显示,在所研究的所有癌组织中Rb蛋白表达水平正常。所有癌组织均未出现细胞周期蛋白D1或CDK4基因的基因组扩增,也没有CDK4的p16结合域发生突变。另外还鉴定出1例p16突变。在所研究的50例癌组织中,共有49例(98%)的Rb/p16通路被破坏,均是通过p16基因的失活。在另一组独立分析的19例胰腺癌中也得到了类似结果。这些数据证明了Rb/p16通路在胰腺癌发生发展中的核心作用。