Institute for Molecular Medicine Finland (FIMM), University of Helsinki, Helsinki, Finland.
MediSapiens Ltd, Erottajankatu 19B, Helsinki, Finland.
Oncogene. 2016 Sep 1;35(35):4601-10. doi: 10.1038/onc.2015.528. Epub 2016 Jan 25.
Almost half of all hereditary breast cancers (BCs) are associated with germ-line mutations in homologous recombination (HR) genes. However, the tumor phenotypes associated with different HR genes vary, making it difficult to define the role of HR in BC predisposition. To distinguish between HR-dependent and -independent features of BCs, we generated a mouse model in which an essential HR gene, Rad51c, is knocked-out specifically in epidermal tissues. Rad51c is one of the key mediators of HR and a well-known BC predisposition gene. Here, we demonstrate that deletion of Rad51c invariably requires inactivation of the Trp53 tumor suppressor (TP53 in humans) to produce mammary carcinomas in 63% of female mice. Nonetheless, loss of Rad51c shortens the latency of Trp53-deficient mouse tumors from 11 to 6 months. Remarkably, the histopathological features of Rad51c-deficient mammary carcinomas, such as expression of hormone receptors and luminal epithelial markers, faithfully recapitulate the histopathology of human RAD51C-mutated BCs. Similar to other BC models, Rad51c/p53 double-mutant mouse mammary tumors also reveal a propensity for genomic instability, but lack the focal amplification of the Met locus or distinct mutational signatures reported for other HR genes. Using the human mammary epithelial cell line MCF10A, we show that deletion of TP53 can rescue RAD51C-deficient cells from radiation-induced cellular senescence, whereas it exacerbates their centrosome amplification and nuclear abnormalities. Altogether, our data indicate that a trend for genomic instability and inactivation of Trp53 are common features of HR-mediated BCs, whereas histopathology and somatic mutation patterns are specific for different HR genes.
几乎一半的遗传性乳腺癌(BC)与同源重组(HR)基因的种系突变有关。然而,不同 HR 基因相关的肿瘤表型不同,使得难以确定 HR 在 BC 易感性中的作用。为了区分 BC 中 HR 依赖性和非依赖性特征,我们生成了一种小鼠模型,其中特定地在表皮组织中敲除了必需的 HR 基因 Rad51c。Rad51c 是 HR 的关键介质之一,也是已知的 BC 易感性基因。在这里,我们证明 Rad51c 的缺失总是需要失活 Trp53 肿瘤抑制基因(人类中的 TP53)才能在 63%的雌性小鼠中产生乳腺肿瘤。尽管如此,Rad51c 的缺失将 Trp53 缺陷型小鼠肿瘤的潜伏期从 11 个月缩短至 6 个月。值得注意的是,Rad51c 缺失型乳腺肿瘤的组织病理学特征,如激素受体和腔上皮标志物的表达,忠实地再现了人类 RAD51C 突变型 BC 的组织病理学特征。与其他 BC 模型类似,Rad51c/p53 双突变型小鼠乳腺肿瘤也显示出基因组不稳定性的倾向,但缺乏 Met 基因座的局灶扩增或其他 HR 基因报道的独特突变特征。使用人乳腺上皮细胞系 MCF10A,我们表明,TP53 的缺失可以使 RAD51C 缺陷细胞从辐射诱导的细胞衰老中恢复,但会加剧它们的中心体扩增和核异常。总的来说,我们的数据表明,基因组不稳定性和 Trp53 失活是 HR 介导的 BC 的共同特征,而组织病理学和体细胞突变模式是特定于不同 HR 基因的。