Department of Molecular Biology, International Institute of Molecular and Cell Biology, 02‑109 Warsaw, Poland.
Institute of Biology, Leiden University, 2333 CC Leiden, The Netherlands.
Int J Oncol. 2019 Apr;54(4):1168-1182. doi: 10.3892/ijo.2019.4723. Epub 2019 Feb 22.
Gain‑of‑function (GOF) mutations in the TP53 gene lead to acquisition of new functions by the mutated tumor suppressor p53 protein. A number of the over‑represented 'hot spot' mutations, including the ones in codons 175, 248 or 273, convey GOF phenotypes. Such phenotypes may include resistance to chemotherapeutics or changes in motility and invasiveness. Whereas the prevalent notion is that the acquisition of the p53 GOF phenotype translates into poorer prognosis for the patient, the analysis of a human somatic p53 mutations dataset demonstrated earlier tumor onset, but decreased frequency and altered location of metastases in patients with the p53‑R248Q allele. Therefore, the GOF activities of p53‑R248Q and p53‑D281G were analyzed in triple negative breast cancer MDA‑MB‑231 and lung adenocarcinoma H1299 cell lines with regard to invasive and metastatic traits. The expression of p53‑D281G increased the motility and invasiveness of the lung cancer cells, but not those of the breast cancer cells. In contrast, the expression of p53‑R248Q decreased the motility and invasiveness of the breast and lung cancer cells in a p53 transactivation‑dependent manner. The intravenous xenotransplantation of MDA‑MB‑231 cells expressing p53‑R248Q into zebrafish embryos resulted in an alteration of the distribution of cancer cells in the body of the fish. In p53‑R248Q‑expressing H1299 cells a decrease in the expression of TCF8/ZEB1 and N‑cadherin was observed, suggesting partial mesenchymal‑to‑epithelial transition. In the two cell lines expressing p53‑R248Q a decrease was noted in the expression of myosin light chain 2, a protein involved in actomyosin‑based motility. To the best of our knowledge, the present study is one of only few reports demonstrating the mutated p53 GOF activity resulting in a decrease of a malignant trait in human cancer.
TP53 基因的获得性功能(GOF)突变导致突变的肿瘤抑制蛋白 p53 获得新的功能。许多代表性的“热点”突变,包括密码子 175、248 或 273 中的突变,赋予 GOF 表型。这种表型可能包括对化疗药物的耐药性或运动和侵袭性的改变。虽然普遍的观点是,获得 p53 GOF 表型会导致患者预后较差,但对人类体细胞 p53 突变数据集的分析表明,携带 p53-R248Q 等位基因的患者肿瘤发病更早,但转移的频率和位置发生了改变。因此,研究人员分析了三阴性乳腺癌 MDA-MB-231 和肺腺癌 H1299 细胞系中 p53-R248Q 和 p53-D281G 的 GOF 活性,研究它们对侵袭和转移特性的影响。p53-D281G 的表达增加了肺癌细胞的运动性和侵袭性,但对乳腺癌细胞没有影响。相反,p53-R248Q 的表达以 p53 反式激活依赖的方式降低了乳腺癌和肺癌细胞的运动性和侵袭性。将表达 p53-R248Q 的 MDA-MB-231 细胞静脉内异种移植到斑马鱼胚胎中,导致癌细胞在鱼体内的分布发生改变。在表达 p53-R248Q 的 H1299 细胞中,观察到 TCF8/ZEB1 和 N-钙粘蛋白表达减少,表明部分间充质-上皮转化。在表达 p53-R248Q 的两种细胞系中,肌球蛋白轻链 2 的表达减少,肌球蛋白轻链 2 是一种参与肌动球蛋白依赖运动的蛋白质。据我们所知,本研究是为数不多的几篇证明突变 p53 GOF 活性导致人类癌症恶性特征降低的报告之一。