Donehower L A, Harvey M, Vogel H, McArthur M J, Montgomery C A, Park S H, Thompson T, Ford R J, Bradley A
Division of Molecular Virology, Baylor College of Medicine, Houston, TX 77030, USA.
Mol Carcinog. 1995 Sep;14(1):16-22. doi: 10.1002/mc.2940140105.
Mice with disrupted germline p53 alleles have been engineered by us and others and have been shown to have enhanced susceptibility to spontaneous tumors of various types. We monitored a large number of p53-deficient mice (p53+/- and p53-/-) and their wild-type littermates (p53+/+) of two different genetic backgrounds (129/Sv and mixed C57BL/6 x 129/Sv) up to 2 yr of age. p53+/- and p53-/- 129/Sv mice show accelerated tumorigenesis rates compared with their p53-deficient counterparts of mixed C57BL/6 x 129/Sv genetic background. The tumor spectra of the two strains of mice are similar except that almost half of 129/Sv p53-/- males develop malignant teratomas, whereas these tumors are rarely observed in C57BL/6 x 129/Sv mice and never in 129/Sv p53+/- males. In the study reported here, we further characterized the lymphomas that arose in the p53-nullizygous mice and found that over three-quarters of the lymphomas were of thymic origin and contained primarily immature (CD4+/CD8+) T-cells, whereas the remainder originated in the spleen and peripheral lymph nodes and were of B-cell type. The high incidence of early-onset lymphomas in the nullizygous mice makes these animals a good lymphoma model, whereas the heterozygous mice may be a useful model for Li-Fraumeni syndrome, a human inherited cancer predisposition.
我们和其他研究人员已经培育出了生殖系p53等位基因缺失的小鼠,这些小鼠对各种类型的自发肿瘤易感性增强。我们监测了大量不同遗传背景(129/Sv和C57BL/6与129/Sv混合背景)的p53基因缺陷小鼠(p53+/-和p53-/-)及其野生型同窝小鼠(p53+/+),直至2岁。与具有C57BL/6与129/Sv混合遗传背景的p53基因缺陷小鼠相比,129/Sv背景的p53+/-和p53-/-小鼠的肿瘤发生速率加快。这两种品系小鼠的肿瘤谱相似,只是129/Sv背景的p53-/-雄性小鼠中近一半会发生恶性畸胎瘤,而在C57BL/6与129/Sv混合背景的小鼠中很少观察到这些肿瘤,在129/Sv背景的p53+/-雄性小鼠中则从未观察到。在本文报道的研究中,我们进一步对p53基因纯合缺失小鼠发生的淋巴瘤进行了特征分析,发现超过四分之三的淋巴瘤起源于胸腺,主要包含未成熟的(CD4+/CD8+)T细胞,而其余的起源于脾脏和外周淋巴结,为B细胞类型。纯合缺失小鼠中早发性淋巴瘤的高发病率使这些动物成为良好的淋巴瘤模型,而异合子小鼠可能是人类遗传性癌症易感性疾病李-弗劳梅尼综合征的有用模型。