The Walter and Eliza Hall Institute of Medical Research, Melbourne, Victoria, Australia.
Cell Death Differ. 2012 Apr;19(4):623-32. doi: 10.1038/cdd.2011.133. Epub 2011 Oct 14.
The tumour suppressor p53 transcriptionally regulates a range of target genes that control cell growth and survival. Mutations of p53 have been implicated in the development of approximately 50% of human cancers, including those instigated by exposure to mutagens. Although numerically rare, cancers can arise as a consequence of inherited mutations, such as in the Li-Fraumeni syndrome, which is caused by mutation of one p53 allele. Gene-targeted mice deficient for p53 have been generated to study this familial cancer syndrome. On a C57BL/6 background, p53-deficient mice develop primarily thymic lymphoma and more rarely sarcoma. Evasion of apoptosis is considered to be essential for neoplastic transformation. As proteins of the Bcl-2 family are the critical regulators of apoptosis, we investigated the role of the pro-survival members Bcl-2, Bcl-x(L) and Bcl-w in cancer development in p53(+/-) and p53(-/-) mice by testing whether ABT-737, a pharmacological inhibitor of these proteins, could prevent or delay tumourigenesis. Our studies showed that ABT-737 prophylaxis only caused a minor delay and reduction in γ-radiation-induced thymic lymphoma development in p53(-/-) mice, but this was accompanied by a concomitant increase in sarcoma. These data show that, collectively, Bcl-2, Bcl-x(L) and Bcl-w have only minor roles in thymic lymphoma development elicited by defects in p53, and this may indicate that Mcl-1 and/or A1 may feature more prominently in this process.
抑癌基因 p53 通过转录调控一系列控制细胞生长和存活的靶基因。p53 的突变与大约 50%的人类癌症的发生有关,包括由诱变剂暴露引起的癌症。尽管数量很少,但癌症也可能是由于遗传突变引起的,例如 Li-Fraumeni 综合征,其由 p53 等位基因的突变引起。为了研究这种家族性癌症综合征,已经生成了基因靶向缺失 p53 的小鼠。在 C57BL/6 背景下,p53 缺失的小鼠主要发展为胸腺淋巴瘤,较少发展为肉瘤。细胞凋亡的逃避被认为是肿瘤转化的必要条件。由于 Bcl-2 家族的蛋白是细胞凋亡的关键调节因子,我们通过测试药理学抑制剂 ABT-737 是否可以预防或延迟 p53(+/-)和 p53(-/-)小鼠中的肿瘤发生,来研究这些存活蛋白 Bcl-2、Bcl-x(L)和 Bcl-w 在 p53 缺失小鼠中的肿瘤发生中的作用。我们的研究表明,ABT-737 预防治疗仅导致 p53(-/-)小鼠的 γ 射线诱导的胸腺淋巴瘤发展出现轻微延迟和减少,但同时伴有肉瘤的增加。这些数据表明,Bcl-2、Bcl-x(L)和 Bcl-w 共同在由 p53 缺陷引起的胸腺淋巴瘤发展中仅发挥次要作用,这可能表明 Mcl-1 和/或 A1 在这个过程中可能更为重要。