Ovarian Cancer Program, Fox Chase Cancer Center, Philadelphia, PA 19111.
Sylvester Comprehensive Cancer Center, Department of Cell Biology, University of Miami Miller School of Medicine, Miami, FL 33136; Department of Medicine, University of California at San Diego, La Jolla, CA 92093.
Neoplasia. 2015 Jan;17(1):89-100. doi: 10.1016/j.neo.2014.11.005.
White spotting variant (Wv) mice are spontaneous mutants attributed to a point mutation in the c-Kit gene, which reduces the tyrosine kinase activity to around 1% and affects the development of melanocytes, mast cells, and germ cells. Homozygous mutant mice are sterile but can live nearly a normal life span. The female Wv mice have a greatly reduced ovarian germ cell and follicle reserve at birth, and the remaining follicles are largely depleted soon after the females reach reproductive stage at around 7 weeks of age. Consequently, ovarian epithelial tumors develop in 100% of Wv females by 3 to 4 months of age. These tumors, called tubular adenomas, are benign but can become invasive in older Wv mice. We tested if additional genetic mutation(s) could convert the benign ovarian epithelial tumors to malignant tumors by crossing the Wv mutant into the Trp53 knockout background. Surprisingly, we found that global deletion of Trp53 suppressed the development of ovarian tubular adenomas in Wv mice. The ovaries of Wv/Wv; Trp53 (-/-) mice were covered by a single layer of surface epithelium and lacked excessive epithelial proliferation. Rather, the ovaries contained a small number of follicles. The presence of ovarian follicles and granulosa cells, as indicated by Pgc7 and inhibin-alpha expression, correlated with the absence of epithelial lesions. A reduction of Pten gene dosage, as in Wv/Wv; Pten (+/-) mice, produced a similar, though less dramatic, phenotype. We conclude that deletion of Trp53 prolongs the survival of ovarian follicles in Wv mice and consequently prevents the proliferation of ovarian epithelial cells and development of ovarian tubular adenomas. The results suggest that various cell types within the ovary communicate and mutually modulate, and an intact tissue environment is required to ensure homeostasis of ovarian surface epithelial cells. Especially, the current finding emphasizes the importance of ovarian follicles in suppressing the hyperplastic growth of ovarian epithelial cells, dominating over the loss of p53.
白色斑点变异(Wv)小鼠是自发突变的,归因于 c-Kit 基因突变,该突变降低了酪氨酸激酶活性约 1%,并影响黑素细胞、肥大细胞和生殖细胞的发育。纯合突变小鼠不育,但可以几乎正常地生活。雌性 Wv 小鼠出生时卵巢生殖细胞和卵泡储备大大减少,而在大约 7 周龄的雌性进入生殖期后,剩余的卵泡很快就被耗尽。因此,Wv 雌性小鼠的卵巢上皮肿瘤在 3 到 4 个月龄时 100%发生。这些肿瘤称为管状腺瘤,是良性的,但在年老的 Wv 小鼠中可能会发生侵袭。我们通过将 Wv 突变体与 Trp53 敲除背景杂交,测试了是否有额外的基因突变可以将良性卵巢上皮肿瘤转化为恶性肿瘤。令人惊讶的是,我们发现 Trp53 的全局缺失抑制了 Wv 小鼠卵巢管状腺瘤的发展。Wv/Wv;Trp53(-/-) 小鼠的卵巢被单层表面上皮覆盖,没有过度的上皮增殖。相反,卵巢中含有少量的卵泡。卵巢滤泡和颗粒细胞的存在,如 Pgc7 和抑制素-α表达所示,与上皮病变的缺失相关。Wv/Wv;Pten(+/-) 小鼠中 Pten 基因剂量的减少产生了类似的、但不太明显的表型。我们得出结论,Trp53 的缺失延长了 Wv 小鼠卵巢滤泡的存活时间,从而防止了卵巢上皮细胞的增殖和卵巢管状腺瘤的发展。结果表明,卵巢内的各种细胞类型相互交流并相互调节,完整的组织环境是确保卵巢表面上皮细胞内稳态所必需的。特别是,目前的发现强调了卵巢滤泡在抑制卵巢上皮细胞过度增生中的重要性,这超过了 p53 的缺失。