Lalle P, Moyret-Lalle C, Wang Q, Vialle J M, Navarro C, Bressac-de Paillerets B, Magaud J P, Ozturk M
Laboratoire d'oncologie moléculaire, INSERM CJF9302, Lyon, France.
Oncogene. 1995 Jun 15;10(12):2447-54.
Increased cancer risk associated with germ-line p53 mutation was linked to a deficit in the ability to maintain genomic stability. Accordingly, normal fibroblasts from cancer-prone individuals accumulate genomic aberrations with concomitant loss of wild-type p53 allele during in vitro culture. We tested whether such changes also occur in EBV-immortalized lymphoblastoid cells. Both normal and p53 germ-line mutant lymphoblastoid cells maintained functional p53 and genomic stability during long term in vitro culture. These unexpected differences between fibroblastic and lymphoblastic cells suggest that phenotypic expression of p53 deficiency is cell type specific. This could contribute to selective tissular localization of tumours observed in patients with Li-Fraumeni syndrome despite the presence of a mutant p53 allele in all cells.
与种系p53突变相关的癌症风险增加与维持基因组稳定性能力的缺陷有关。因此，来自癌症易感个体的正常成纤维细胞在体外培养过程中会积累基因组畸变，并伴随野生型p53等位基因的丢失。我们测试了这些变化是否也发生在EB病毒永生化的淋巴母细胞中。正常和p53种系突变的淋巴母细胞在长期体外培养过程中都维持了功能性p53和基因组稳定性。成纤维细胞和淋巴细胞之间这些意外的差异表明，p53缺陷的表型表达具有细胞类型特异性。这可能有助于解释在李-佛美尼综合征患者中观察到的肿瘤选择性组织定位现象，尽管所有细胞中都存在突变的p53等位基因。
